Early benefit assessment: Detailed analysis of all G-BA resolutions

AMNOG.de provides current, well-founded analyses of all early benefit assessments and an overview of the negotiated reimbursement prices in Germany. It is an indispensable tool for HTA, market access and pricing managers.
 
Serplulimab (5) Hetronifly® Accord Healthcare GmbH Oncological diseases Small-cell lung cancer, in combination with carboplatin and etoposide, first-line treatment n.d. active procedure
Serplulimab (4) Hetronifly® Accord Healthcare GmbH Oncological diseases Non-small cell lung cancer, squamous cell, first-line treatment, combination with carboplatin and nab-paclitaxel n.d. active procedure
Pegvaliase (2) Palynziq® BioMarin Deutschland GmbH Metabolic diseases Phenylketonuria; 12 to < 16 years n.d. active procedure Orphan
Enfortumab Vedotin (3) Padcev® Astellas Pharma GmbH Oncological diseases Muscle-invasive bladder cancer, neoadjuvant/adjuvant therapy following cystectomy, in combination with pembrolizumab n.d. active procedure
Sacituzumab govitecan (3) Trodelvy® Gilead Sciences GmbH Oncological diseases Triple-negative breast cancer, first-line treatment n.d. active procedure
Glecaprevir / Pibrentasvir (4) Maviret® AbbVie Deutschland GmbH & Co. KG Infectious diseases Acute hepatitis C virus infection, ≥ 3 years n.d. active procedure
Onasemnogen abeparvovec (1) Itvisma® Novartis Pharma GmbH Nervous system diseases 5q-associated spinal muscular atrophy with ≥ 4 copies of the SMN2 gene and SMA type 2, type 3, type 4 or of undefined type, aged ≥ 2 years n.d. active procedure
Risankizumab (5) Skyrizi® AbbVie Deutschland GmbH & Co. KG Skin diseases Moderate to severe plaque psoriasis; 6 to < 18 years n.d. active procedure
Serplulimab (3) Hetronifly® Accord Healthcare GmbH Oncological diseases Squamous cell carcinoma of the oesophagus, PD-L1 with CPS ≥ 5, first-line treatment, in combination with fluoropyrimidine- and platinum-based chemotherapy n.d. active procedure
Serplulimab (2) Hetronifly® Accord Healthcare GmbH Oncological diseases Non-small cell lung cancer, non-squamous, EGFR-, ALK- and ROS-negative, first-line treatment, combination with carboplatin and pemetrexed n.d. active procedure
Durvalumab (12) Imfinzi® AstraZeneca GmbH Oncological diseases Adenocarcinoma of the stomach or the gastro-oesophageal junction; neoadjuvant and adjuvant combination with FLOT chemotherapy followed by adjuvant monotherapy n.d. active procedure
Decitabin / Cedazuridin (2) Inaqovi® Otsuka Pharma GmbH Oncological diseases Acute myeloid leukaemia, first-line treatment, combination with venetoclax n.d. active procedure
Encorafenib (4) Braftovi® Pierre Fabre Pharma GmbH Oncological diseases Colorectal cancer with a BRAF V600E mutation; first-line treatment; combination with cetuximab and FOLFOX n.d. active procedure
Benralizumab (3) Fasenra® AstraZeneca GmbH Hematopoietic diseases Hypereosinophilic syndrome, aged ≥ 12 years, weighing ≥ 35 kg n.d. active procedure
Epcoritamab (4) Tepkinly® AbbVie Deutschland GmbH Oncological diseases Follicular lymphoma, relapsed or refractory, in combination with lenalidomide and rituximab n.d. active procedure
Zuranolon (1) Zurzuvae® Biogen GmbH Mental illnesses Postnatal depression n.d. active procedure
Atidarsagen autotemcel OTL-200 (2) Libmeldy® Orchard Therapeutics GmbH Metabolic diseases Metachromatic leukodystrophy with a biallelic mutation in the ARSA gene n.d. active procedure Orphan
Deutivacaftor / Tezacaftor / Vanzacaftor (2) Alyftrek® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis, at least one non-Class I mutation, aged ≥ 6 years n.d. active procedure
Leniolisib (1) Joenja® Pharming Group BV Hematopoietic diseases Activated phosphoinositide-3-kinase delta syndrome, aged ≥ 12 years, weighing ≥ 45 kg n.d. active procedure Orphan
Burosumab (7) Crysvita® Kyowa Kirin GmbH Metabolic diseases X-linked hypophosphataemia; ≥ 1 month to < 1 year n.d. active procedure Orphan (turnover limit)
Lurbinectedin (1) Zepzelca® Pharma Mar S.A. Oncological diseases Small-cell lung cancer, extensive-stage, maintenance therapy, in combination with atezolizumab n.d. active procedure Orphan
Venetoclax (7) Venclyxto® AbbVie Deutschland GmbH & Co. KG Oncological diseases Chronic lymphocytic leukaemia, first-line treatment, combination of acalabrutinib with or without obinutuzumab n.d. active procedure
Venetoclax (6) Venclyxto® AbbVie Deutschland GmbH & Co. KG Oncological diseases Chronic lymphocytic leukaemia, first-line treatment, combination with ibrutinib n.d. active procedure
Tarlatamab (1) Imdylltra® Amgen GmbH Oncological diseases Advanced small-cell lung cancer, previously treated n.d. active procedure Orphan
Atogepant (2) Aquipta® AbbVie Deutschland GmbH & Co. KG Nervous system diseases Acute treatment of migraine n.d. active procedure
Ganaxolon (1) Ztalmy® Immedica Pharma Germany GmbH Nervous system diseases CDKL5 deficiency, adjunctive therapy, 2 to < 18 years n.d. active procedure Orphan
Dorocubicel / nicht-expandierte allogene CD34- Zellen aus der Nabelschnur (1) Zemcelpro® Cordex Biologics International Ltd Oncological diseases Haematological malignancies, allogeneic haematopoietic stem cell transplantation indicated, no donor available n.d. active procedure
Vamorolon (2) Agamree® Santhera Pharmaceuticals GmbH Musculoskeletal system diseases Duchenne muscular dystrophy, aged ≥ 2 to < 4 years n.d. active procedure Orphan
Selpercatinib (12) Retsevmo® Lilly Deutschland GmbH Oncological diseases Thyroid carcinoma, RET-fusion-positive, refractory to radioactive iodine, ≥ 2 years n.d. active procedure
Selpercatinib (11) Retsevmo® Lilly Deutschland GmbH Oncological diseases Solid tumours, RET-fusion-positive, aged ≥ 2 to < 18 years n.d. active procedure
Selpercatinib (10) Retsevmo® Lilly Deutschland GmbH Oncological diseases RET-mutated medullary thyroid carcinoma (MTC), ≥ 2 years n.d. active procedure
Mexiletin (2) Namuscla® Lupin Europe GmbH Nervous system diseases Myotonia, 6 to ≤ 17 years, ≥ 20 kg n.d. active procedure Orphan
Deucravacitinib (2) Sotyktu® Bristol-Myers Squibb GmbH & Co. KGaA Skin diseases Psoriatic arthritis, as monotherapy or in combination with methotrexate n.d. active procedure
Nivolumab / Relatlimab (1) Opdualag® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, PD-L1 expression < 1 per cent, advanced, first-line treatment, ≥ 12 years n.d. active procedure
Remibrutinib (1) Rhapsido® Novartis Pharma GmbH Skin diseases Chronic spontaneous urticaria n.d. active procedure
Clascoteron (1) Winlevi® InfectoPharm Arzneimittel und Consilium GmbH Skin diseases Acne vulgaris; ≥ 12 years n.d. active procedure
Marstacimab (4) Hympavzi® Pfizer Pharma GmbH Hematopoietic diseases Haemophilia B, aged ≥ 12 years, with factor IX inhibitors n.d. active procedure
Marstacimab (3) Hympavzi® Pfizer Pharma GmbH Hematopoietic diseases Haemophilia A, aged ≥ 12 years, with factor VIII inhibitors n.d. active procedure
Blinatumomab (14) Blincyto® Amgen GmbH Oncological diseases Acute lymphocytic B-cell leukaemia, Ph-, CD19+, newly diagnosed, adults n.d. active procedure Orphan (turnover limit)
Blinatumomab (13) Blincyto® Amgen GmbH Oncological diseases Acute lymphocytic B-cell leukaemia, Ph-, CD19+, high-risk first relapse, children aged ≥1 month n.d. active procedure Orphan (turnover limit)
Blinatumomab (12) Blincyto® Amgen GmbH Oncological diseases Acute lymphocytic B-cell leukaemia, Ph-, CD19+, MRD-positive, adults n.d. active procedure Orphan (turnover limit)
Blinatumomab (11) Blincyto® Amgen GmbH Oncological diseases Acute lymphocytic B-cell leukaemia, Ph-, CD19+, relapsed/refractory, following ≥ 2 prior treatments or following allogeneic haematopoietic stem cell transplantation, children aged ≥ 1 month n.d. active procedure Orphan (turnover limit)
Blinatumomab (10) Blincyto® Amgen GmbH Oncological diseases Acute lymphocytic B-cell leukaemia, CD19+, relapsed or refractory, adults n.d. active procedure Orphan (turnover limit)
Inotuzumab ozogamicin (2) Besponsa® Pfizer Pharma GmbH Oncological diseases Acute lymphoblastic B-cell leukaemia, relapsed/refractory, following allogeneic haematopoietic stem cell transplantation or following a very high-risk relapse or following ≥ 2 prior treatments, CD22+, paediatric patients aged ≥ 1 year n.d. active procedure Orphan
Aficamten (1) Myqorzo® Cytokinetics Germany GmbH Cardiovascular diseases Hypertrophic obstructive cardiomyopathy n.d. active procedure
Asciminib (4) Scemblix® Novartis Pharma GmbH Oncological diseases Chronic myeloid leukaemia, Ph+, CML-CP with T315I mutation, previously treated n.d. active procedure Orphan (turnover limit)
Pretomanid (1) Dovprela® Viatris Germany GmbH Infectious diseases Tuberculosis n.d. active procedure Orphan
Clesrovimab (1) Enflonsia® MSD Sharp & Dohme GmbH Infectious diseases Secondary prevention of RSV infections in children during their first RSV season n.d. active procedure
Zolbetuximab (2) Vyloy® Astellas Pharma GmbH Oncological diseases Adenocarcinoma of the stomach or the gastro-oesophageal junction, HER2-negative, first-line treatment, combination with fluoropyrimidine- and platinum-based chemotherapy n.d. active procedure Orphan (turnover limit)
Tovorafenib (1) Ojemda® Ipsen Pharma GmbH Oncological diseases Malignant glioma, low-grade with a BRAF fusion or rearrangement or a BRAF V600 mutation, following at least one prior line of treatment, ≥ 6 months n.d. active procedure Orphan
Dupilumab (13) Dupixent® Sanofi-Aventis Deutschland GmbH Skin diseases Chronic spontaneous urticaria, aged ≥ 2 years to < 12 years n.d. active procedure
Dupilumab (12) Dupixent® Sanofi-Aventis Deutschland GmbH Skin diseases Chronic spontaneous urticaria, aged ≥ 12 years n.d. active procedure
Belumosudil (1) Rezurock® Sanofi-Aventis Deutschland GmbH Oncological diseases Chronic graft-versus-host disease (cGvHD), aged ≥ 12 years, weighing ≥ 40 kg n.d. active procedure Orphan
Olipudase alfa (2) Xenpozyme® Sanofi-Aventis Deutschland GmbH Metabolic diseases Acid sphingomyelinase deficiency (ASMD) type A/B or type B n.d. active procedure Orphan (turnover limit)
Finerenon (3) Kerendia® Bayer Vital GmbH Cardiovascular diseases Chronic heart failure with a left ventricular ejection fraction (LVEF) > 40% n.d. active procedure
Depemokimab (2) Exdensur® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Chronic rhinosinusitis with nasal polyps n.d. active procedure
Depemokimab (1) Exdensur® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Bronchial asthma, ≥ 12 years n.d. active procedure
Elinzanetant (2) Lynkuet® Bayer Vital GmbH Other diseases Vasomotor symptoms associated with breast cancer n.d. active procedure
Elinzanetant (1) Lynkuet® Bayer Vital GmbH Other diseases Vasomotor symptoms associated with the menopause n.d. active procedure
Retifanlimab (2) Zynyz® Incyte Biosciences Germany GmbH Oncological diseases Squamous cell carcinoma of the anal canal; first-line treatment n.d. active procedure
Retifanlimab (1) Zynyz® Incyte Biosciences Germany GmbH Oncological diseases Merkel cell carcinoma, metastatic or recurrent, monotherapy, first-line treatment n.d. active procedure
Belantamab-Mafodotin (4) Blenrep® GlaxoSmithKline GmbH & Co. KG Oncological diseases Multiple myeloma, relapsed or refractory, following at least one prior course of treatment; combination with pomalidomide and dexamethasone n.d. active procedure
Belantamab-Mafodotin (3) Blenrep® GlaxoSmithKline GmbH & Co. KG Oncological diseases Multiple myeloma, relapsed or refractory, following at least one prior course of treatment; combination therapy with bortezomib and dexamethasone n.d. active procedure
Niraparib / Abirateronacetat (2) Akeega® Janssen-Cilag GmbH Oncological diseases Metastatic, hormone-sensitive prostate cancer; BRCA1/2 mutations; combination with prednis(ol)one and androgen deprivation therapy n.d. active procedure
Imlunestrant (1) Inluriyo® Lilly Deutschland GmbH Oncological diseases Breast cancer, ER+, HER2-, with an ESR1 mutation, progressive following prior endocrine therapy n.d. active procedure
Inebilizumab (3) Uplizna® Amgen GmbH Nervous system diseases Myasthenia gravis, AChR antibody-positive, MuSK antibody-positive n.d. active procedure
Inebilizumab (2) Uplizna® Amgen GmbH Other diseases Immunoglobulin G4-associated disease n.d. active procedure
Mepolizumab (6) Nucala® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases COPD n.d. active procedure
Gadopiclenol (2) Elucirem® Guerbet GmbH Oncological diseases Contrast-enhanced magnetic resonance imaging, 0 to < 2 years n.d. active procedure
Ivosidenib (4) Tibsovo® Servier Deutschland GmbH Oncological diseases Cholangiocarcinoma with an IDH1 R132 mutation, following at least one prior course of treatment n.d. active procedure Orphan (turnover limit)
Ivosidenib (3) Tibsovo® Servier Deutschland GmbH Oncological diseases Acute myeloid leukaemia with the IDH1 R132 mutation, first-line treatment, in combination with azacitidine n.d. active procedure Orphan (turnover limit)
Abemaciclib (7) Verzenios® Lilly Deutschland GmbH Oncological diseases Breast cancer, HR+, HER2-, early-stage with a high risk of recurrence, adjuvant therapy, combination with endocrine therapy n.d. active procedure
Colchicin (1) Colxi® Apontis Pharma Deutschland GmbH & Co. KG Cardiovascular diseases For the prevention of ischaemic cardiovascular events in patients with atherosclerotic coronary heart disease following a myocardial infarction n.d. active procedure
Tirzepatid (2) Mounjaro® Lilly Deutschland GmbH Metabolic diseases Type 2 diabetes mellitus, aged ≥ 10 to ≤ 17 years n.d. active procedure
Teprotumumab (1) Tepezza® Amgen GmbH Eye diseases Endocrine orbitopathy n.d. active procedure
Donidalorsen (1) Dawnzera® Otsuka Pharma GmbH Other diseases Hereditary angioedema, prophylaxis, ≥ 12 years n.d. active procedure
Zanidatamab (1) Ziihera® Jazz Pharmaceuticals Ireland Limited Oncological diseases Biliary carcinoma, HER2+ (IHC3+), previously treated 30–160 100% Hint for non-quantifiable additional benefit Orphan
Sotatercept (2) Winrevair® MSD Sharp & Dohme GmbH Cardiovascular diseases Pulmonary arterial hypertension, WHO functional class IV 35–130 100% Hint for non-quantifiable additional benefit Orphan
Teplizumab (1) Teizeild® Sanofi-Aventis Deutschland GmbH Metabolic diseases Type 1 diabetes mellitus, aged ≥ 8 years 57,000 100% Hint for non-quantifiable additional benefit
Pegcetacoplan (4) Aspaveli® Swedish Orphan Biovitrum AB Genitourinary system diseases Primary immune-complex-mediated membranoproliferative glomerulonephritis, ≥ 12 years 75–180 100% Hint for non-quantifiable additional benefit Orphan
Pegcetacoplan (3) Aspaveli® Swedish Orphan Biovitrum AB Genitourinary system diseases Complement-3 glomerulopathy, ≥ 12 years 180–370 100% Hint for non-quantifiable additional benefit Orphan
Selumetinib (5) Koselugo® Alexion Pharma Germany GmbH Oncological diseases Neurofibromatosis type 1 (≥ 1 to < 3 years) 55–95 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Imipenem / Cilastatin / Relebactam (3) Recarbrio® MSD Sharp & Dohme GmbH Infectious diseases Bacterial infections, multiple therapeutic indications, under 18 years of age 519–1,299 100% additional benefit considered proven
Eravacyclin (2) Xerava® Paion Pharma GmbH Infectious diseases Complicated intra-abdominal infections (cIAI), aged ≥ 12 to < 18 years, weighing ≥ 50 kg 175–440 100% additional benefit considered proven
Mirvetuximab-Soravtansin (2) Elahere® AbbVie Deutschland GmbH & Co. KG Oncological diseases Ovarian cancer, fallopian tube cancer or primary peritoneal cancer, FRα-positive, platinum-resistant, following 1 to 3 prior treatments 630–1,300 100% Indication of considerable additional benefit Orphan (turnover limit)
Tafasitamab (2) Minjuvi® Incyte Biosciences Germany GmbH Oncological diseases Follicular lymphoma, following ≥ 1 prior line of treatment, in combination with lenalidomide and rituximab 1,050–2,350 100% Hint for non-quantifiable additional benefit Orphan
Toripalimab (2) Loqtorzi® LEO Pharma GmbH Oncological diseases Squamous cell carcinoma of the oesophagus, first-line treatment, in combination with cisplatin and paclitaxel 2,600 100% additional benefit not proven
Toripalimab (1) Loqtorzi® LEO Pharma GmbH Oncological diseases Recurrent or metastatic nasopharyngeal carcinoma (NPC), first-line treatment, in combination with cisplatin and gemcitabine 70–120 100% additional benefit not proven
Guselkumab (5) Tremfya® Johnson & Johnson Skin diseases Moderate to severe plaque psoriasis; 6 to < 18 years 360–440 100% additional benefit not proven
Remimazolam (2) Byfavo® Paion Pharma GmbH Other diseases Induction and maintenance of general anaesthesia 12,000,000 100% additional benefit not proven
Remimazolam (1) Byfavo® Paion Pharma GmbH Other diseases Procedural sedation 2,620,000–2,730,000 100% additional benefit not proven
Nipocalimab (1) Imaavy® Johnson & Johnson Nervous system diseases Myasthenia gravis, AChR antibody-positive, MuSK antibody-positive, aged ≥ 12 years 6,570–19,990 100% additional benefit not proven
Selpercatinib (9) Retsevmo® Lilly Deutschland GmbH Oncological diseases Non-small cell lung cancer, RET-fusion+, first-line treatment 155–270 100% additional benefit not proven
Nivolumab (33) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Urothelial carcinoma, PD-L1 expression ≥ 1 %, adjuvant therapy 680–830 100% Hint for considerable additional benefit
Lisocabtagen maraleucel (4) Breyanzi® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Mantle cell lymphoma, following ≥ 2 prior treatments 105–150 100% additional benefit not proven
Nirmatrelvir / Ritonavir (2) Paxlovid® Pfizer Pharma GmbH Infectious diseases COVID-19, no need for supplemental oxygen, aged ≥ 6 years to < 18 years, weighing ≥ 20 kg 40–230 100% additional benefit not proven
Cemiplimab (6) Libtayo® Regeneron GmbH Oncological diseases Cutaneous squamous cell carcinoma, following resection and radiotherapy, adjuvant therapy 690–1,420 100% Indication of non-quantifiable additional benefit
Iptacopan (4) Fabhalta® Novartis Pharma GmbH Genitourinary system diseases Complement-3 glomerulopathy 110–230 100% additional benefit not proven Orphan (turnover limit)
Iptacopan (3) Fabhalta® Novartis Pharma GmbH Hematopoietic diseases Paroxysmal nocturnal haemoglobinuria 290–945 100% additional benefit not proven Orphan (turnover limit)
Olezarsen (1) Tryngolza® Swedish Orphan Biovitrum GmbH Metabolic diseases Chylomicronemia syndrome 60–130 100% Indication of minor additional benefit Orphan
Asciminib (3) Scemblix® Novartis Pharma GmbH Oncological diseases Chronic myeloid leukaemia, Philadelphia chromosome-positive, chronic phase (Ph+ CML-CP) 7,130–7,330 80% Hint for considerable additional benefit Orphan (turnover limit)
Pembrolizumab (41) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Resectable locally advanced squamous cell carcinoma of the head and neck, PD-L1 expression ≥ 1 per cent, neoadjuvant and adjuvant therapy, in combination with radiotherapy with or without concomitant cisplatin therapy 2,550–4,240 86% Hint for minor additional benefit
Diflunisal (1) Attrogy® Purpose Pharma International AB Metabolic diseases Hereditary transthyretin amyloidosis with polyneuropathy (stage 1 or 2) 360 100% additional benefit not proven
Selumetinib (4) Koselugo® Alexion Pharma Germany GmbH Oncological diseases Neurofibromatosis type 1 (aged 18 years and over) 515–920 100% Indication of minor additional benefit Orphan (turnover limit)
Selumetinib (3) Koselugo® Alexion Pharma Germany GmbH Oncological diseases Neurofibromatosis type 1 (aged ≥ 3 to < 18 years) 515–920 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Tezepelumab (2) Tezspire® AstraZeneca GmbH Respiratory system diseases Chronic rhinosinusitis with nasal polyps 10,500–12,600 100% Hint for non-quantifiable additional benefit
Vorasidenib (1) Voranigo® Servier Deutschland GmbH Oncological diseases Astrocytoma or oligodendroglioma, grade 2, IDH1-R132 or IDH2-R172 mutation, following surgical intervention, aged ≥ 12 years and weighing ≥ 40 kg 380–800 100% Hint for non-quantifiable additional benefit Orphan
Avapritinib (4) Ayvakyt® Blueprint Medicines (Germany) GmbH Oncological diseases Indolent systemic mastocytosis (ISM) 715–1,000 100% Hint for minor additional benefit Orphan (turnover limit)
Avapritinib (6) Ayvakyt® Blueprint Medicines (Germany) GmbH Oncological diseases Gastrointestinal stromal tumours 5–60 100% additional benefit not proven Orphan (turnover limit)
Avapritinib (5) Ayvakyt® Blueprint Medicines (Germany) GmbH Oncological diseases Advanced systemic mastocytosis, following at least one prior course of treatment 260–680 100% additional benefit not proven Orphan (turnover limit)
Momelotinib (2) Omjjara® GlaxoSmithKline GmbH & Co. KG Oncological diseases Myelofibrosis 680–2,680 100% additional benefit not proven Orphan (turnover limit)
Donanemab (1) Kisunla® Lilly Deutschland GmbH Nervous system diseases Early-onset Alzheimer’s disease 131,000–440,000 100% additional benefit not proven
Vimseltinib (1) Romvimza® Deciphera Pharmaceuticals (Netherlands) B.V. Oncological diseases Symptomatic tenosynovial giant cell tumours 160–1,140 100% Indication of considerable additional benefit Orphan
Nirogacestat (1) Ogsiveo® SpringWorks Therapeutics Ireland Limited Oncological diseases Desmoid tumour, advanced 350–630 100% Hint for minor additional benefit Orphan
Sebetralstat (1) Ekterly® KalVista Pharmaceuticals Germany GmbH Other diseases Hereditary angioedema, acute treatment, ≥ 12 years 1,000–1,100 100% Hint for non-quantifiable additional benefit Orphan
Trastuzumab deruxtecan (7) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases HER2-positive adenocarcinoma of the stomach or the gastro-oesophageal junction, following trastuzumab-based therapy 360–600 100% Indication of minor additional benefit
Ixekizumab (6) Taltz® Lilly Deutschland GmbH Musculoskeletal system diseases Enthesitis-associated arthritis, ≥ 6 years 240–290 100% additional benefit not proven
Tislelizumab (9) Tevimbra® BeOne Medicines Germany GmbH Oncological diseases Non-small cell lung cancer, high risk of recurrence, neoadjuvant and adjuvant therapy, monotherapy or in combination with platinum-based chemotherapy 5,090–5,780 100% additional benefit not proven
Tislelizumab (8) Tevimbra® BeOne Medicines Germany GmbH Oncological diseases Recurrent or metastatic nasopharyngeal carcinoma (NPC), first-line treatment, in combination with gemcitabine and cisplatin 70–120 100% additional benefit not proven
Tislelizumab (7) Tevimbra® BeOne Medicines Germany GmbH Oncological diseases Small cell lung cancer, first-line treatment, in combination with etoposide and platinum-based chemotherapy 3,820–8,124 100% additional benefit not proven
Mirdametinib (1) Ezmekly® SpringWorks Therapeutics Ireland Limited Oncological diseases Plexiform neurofibromas (PN), neurofibromatosis type 1 (NF1); ≥ 2 years 515–920 100% Hint for non-quantifiable additional benefit Orphan
Linvoseltamab (1) Lynozyfic® Regeneron GmbH Oncological diseases Multiple myeloma, at least 3 prior treatments, monotherapy 330–340 100% additional benefit not proven
Ixekizumab (5) Taltz® Lilly Deutschland GmbH Skin diseases Juvenile psoriatic arthritis, aged ≥ 6 years 120–180 100% additional benefit not proven
Concizumab (4) Alhemo® Novo Nordisk GmbH Hematopoietic diseases Haemophilia B, aged ≥ 12 years, without factor IX inhibitors 290–490 100% additional benefit not proven
Concizumab (3) Alhemo® Novo Nordisk GmbH Hematopoietic diseases Haemophilia A, aged ≥ 12 years, without factor VIII inhibitors 1,800–2,000 100% additional benefit not proven
Resmetirom (1) Rezdiffra® Madrigal Pharmaceuticals EU Limited Digestive system diseases Metabolic dysfunction-associated steatohepatitis (MASH), non-cirrhotic 15,000–24,000 100% additional benefit not proven
Avacopan (2) Tavneos® Fresenius Medical Care Nephrologica Deutschland Musculoskeletal system diseases Granulomatosis with polyangiitis or microscopic polyangiitis, in combination with rituximab or cyclophosphamide 2,330–2,850 100% additional benefit not proven Orphan (turnover limit)
Bulevirtid (4) Hepcludex® Gilead Sciences GmbH Infectious diseases Hepatitis delta virus (HDV) infection, HDV RNA-positive, aged ≥ 3 years, body weight ≥ 10 kg 400–3,202 100% additional benefit not proven Orphan (turnover limit)
Tisotumab vedotin (1) Tivdak® Genmab Germany GmbH Oncological diseases Cervical cancer, previously treated 381–1,452 100% additional benefit not proven
Lecanemab (1) Leqembi® Eisai GmbH Nervous system diseases Early-onset Alzheimer’s disease 131,000–440,000 100% additional benefit not proven
Daratumumab (15) Darzalex® Janssen-Cilag GmbH Oncological diseases Smouldering multiple myeloma (SMM) 160–325 100% Hint for minor additional benefit Orphan (turnover limit)
Daratumumab (14) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma, unsuitable for stem cell transplantation, in combination with bortezomib, lenalidomide and dexamethasone 3,450–3,680 100% Hint for minor additional benefit Orphan (turnover limit)
Isatuximab (4) Sarclisa® Sanofi-Aventis Deutschland GmbH Oncological diseases Multiple myeloma, first-line treatment, suitable for stem cell transplantation, in combination with bortezomib, lenalidomide and dexamethasone 1,750–1,910 100% additional benefit not proven
Darolutamid (3) Nubeqa® Bayer Vital GmbH Oncological diseases Metastatic, hormone-sensitive prostate cancer, in combination with androgen deprivation therapy 2,590–3,640 100% additional benefit not proven
Beremagen geperpavec (1) Vyjuvek® Krystal Biotech Skin diseases Wound treatment in dystrophic epidermolysis bullosa, all age groups 240–900 100% Hint for non-quantifiable additional benefit Orphan
Inavolisib (1) Itovebi® Roche Pharma AG Oncological diseases Breast cancer, PIK3CA-mutated, ER+, HER2-, locally advanced or metastatic, recurrence < 12 months after adjuvant endocrine therapy, in combination with palbociclib and fulvestrant 868–3,625 50% Hint for considerable additional benefit
Efgartigimod alfa (3) Vyvgart® Argenx Germany GmbH Nervous system diseases Chronic inflammatory demyelinating polyneuropathy, in patients who have received prior treatment 50–260 100% additional benefit not proven Orphan (turnover limit)
Odronextamab (2) Ordspono® Regeneron GmbH Oncological diseases Follicular lymphoma, following ≥ 2 prior treatments 370–840 100% additional benefit not proven
Odronextamab (1) Ordspono® Regeneron GmbH Oncological diseases Diffuse large B-cell lymphoma (DLBCL), following ≥ 2 prior treatments 960–2,130 100% additional benefit not proven
Durvalumab (11) Imfinzi® AstraZeneca GmbH Oncological diseases Muscle-invasive bladder cancer (MIBC), neoadjuvant/adjuvant therapy following cystectomy, in combination with gemcitabine and cisplatin 4,310–5,730 100% Indication of minor additional benefit
Durvalumab (10) Imfinzi® AstraZeneca GmbH Oncological diseases Non-advanced small cell lung cancer, following platinum-based chemoradiotherapy, monotherapy 670–1,750 100% Indication of considerable additional benefit
Durvalumab (9) Imfinzi® AstraZeneca GmbH Oncological diseases Non-small cell lung cancer, EGFR/ALK-negative, neoadjuvant/adjuvant therapy, in combination with platinum-based chemotherapy 4,540–4,660 100% additional benefit not proven
Deutivacaftor / Tezacaftor / Vanzacaftor (1) Alyftrek® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, at least one non-Class I mutation, aged ≥ 6 years n.d. discontinued Orphan
Sepiapterin (1) Sephience® PTC Therapeutics International Limited Metabolic diseases Hyperphenylalaninaemia in phenylketonuria 4,700–6,800 100% Hint for non-quantifiable additional benefit Orphan
Givinostat (1) Duvyzat® ITF Pharma GmbH Musculoskeletal system diseases Duchenne muscular dystrophy, ≥ 6 years, in combination with a corticosteroid 270–400 100% Hint for non-quantifiable additional benefit Orphan
Remdesivir (5) Veklury® Gilead Sciences GmbH Infectious diseases COVID-19, no need for supplemental oxygen, ≥ 4 weeks, ≥ 3 kg to <40 kg 23 100% additional benefit not proven
Vutrisiran (2) Amvuttra® Alnylam Germany GmbH Metabolic diseases Wild-type or hereditary transthyretin amyloidosis with cardiomyopathy 1,760–2,120 100% additional benefit not proven Orphan
Andexanet alfa (2) Ondexxya® AstraZeneca GmbH Hematopoietic diseases Repeal of anticoagulant therapy 9,900–32,600 50% additional benefit not proven
Acalabrutinib (7) Calquence® AstraZeneca GmbH Oncological diseases Chronic lymphocytic leukaemia, first-line treatment, in combination with venetoclax and obinutuzumab 3,200 100% additional benefit not proven
Omaveloxolon (2) Skyclarys® Biogen GmbH Nervous system diseases Friedreich’s ataxia, aged 16 years and over 990 100% additional benefit not proven Orphan (turnover limit)
Nivolumab (32) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Cancer of the oesophagus or gastro-oesophageal junction, in previously treated patients, as adjuvant therapy 580–910 100% Hint for minor additional benefit
Acalabrutinib (6) Calquence® AstraZeneca GmbH Oncological diseases Mantle cell lymphoma, no prior BTKi treatment, relapsed or refractory, monotherapy 220 100% additional benefit not proven
Acalabrutinib (5) Calquence® AstraZeneca GmbH Oncological diseases Mantle cell lymphoma, autologous stem cell transplant not suitable, first-line treatment, in combination with bendamustine and rituximab 220–460 100% additional benefit not proven
Acalabrutinib (4) Calquence® AstraZeneca GmbH Oncological diseases Chronic lymphocytic leukaemia, first-line treatment, in combination with venetoclax 3,200 100% additional benefit not proven
Nivolumab (29) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Colorectal cancer with MSI-H or dMMR, first-line treatment, in combination with ipilimumab 560–1,800 100% additional benefit not proven
Nivolumab (31) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Non-small cell lung cancer, PD-L1 expression ≥ 1 per cent, neoadjuvant and adjuvant therapy, monotherapy or in combination with platinum-based chemotherapy 3,240–3,680 100% additional benefit not proven
Nivolumab (30) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Unresectable or advanced hepatocellular carcinoma, first-line treatment, in combination with ipilimumab 1,900–5,470 100% additional benefit not proven
Guselkumab (4) Tremfya® Johnson & Johnson Digestive system diseases Crohn’s disease, previously treated 12,600–78,300 46% Indication of minor additional benefit
Guselkumab (3) Tremfya® Johnson & Johnson Digestive system diseases Ulcerative colitis, previously treated 29,100 100% additional benefit not proven
Dalbavancin (3) Xydalba® Advanz Pharma Germany GmbH Infectious diseases Acute bacterial skin and soft tissue infections (ABSSSI), from birth to < 3 months 16–38 100% additional benefit considered proven
Selpercatinib (8) Retsevmo® Lilly Deutschland GmbH Oncological diseases Thyroid carcinoma, RET-mutated, monotherapy, from 12 years of age 40–170 100% Indication of major additional benefit
Datopotamab deruxtecan (1) Datroway® Daiichi Sankyo Deutschland GmbH Oncological diseases HR+, HER2- breast cancer, following at least one prior course of treatment 4,140–14,160 100% additional benefit not proven
Rimegepant (2) Vydura® Pfizer Pharma GmbH Nervous system diseases Migraine prophylaxis 1,386,900–1,726,200 100% additional benefit not proven
Rimegepant (1) Vydura® Pfizer Pharma GmbH Nervous system diseases Acute treatment of migraine 2,825,000 100% additional benefit not proven
Asciminib (2) Scemblix® Novartis Pharma GmbH Oncological diseases Chronic myeloid leukaemia, Ph+, following ≥ 2 prior treatments 1,500–1,730 50% Indication of minor additional benefit Orphan (turnover limit)
Glofitamab (3) Columvi® Roche Pharma AG Oncological diseases Diffuse large B-cell lymphoma (DLBCL), following ≥ 2 prior treatments 960–2,130 100% additional benefit not proven
Glofitamab (2) Columvi® Roche Pharma AG Oncological diseases Diffuse large B-cell lymphoma, relapsed or refractory, in combination with gemcitabine and oxaliplatin; autologous stem cell transplantation is not suitable 2,230–3,550 100% additional benefit not proven
Letermovir (5) Prevymis® MSD Sharp & Dohme GmbH Infectious diseases CMV infection, prophylaxis following kidney transplantation, < 18 years of age, ≥ 40 kg 13 100% additional benefit not proven Orphan (turnover limit)
Letermovir (4) Prevymis® MSD Sharp & Dohme GmbH Infectious diseases CMV reactivation/disease, prophylaxis following stem cell transplantation, < 18 years, ≥ 5 kg 160–240 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Upadacitinib (8) Rinvoq® AbbVie Deutschland GmbH & Co. KG Musculoskeletal system diseases Giant cell arteritis 13,100–15,900 20% Hint for minor additional benefit
RdESAT-6 / rCFP-10 (1) Siiltibcy® Serum Life Science Europe GmbH Infectious diseases Other diagnostic agents n.d. exempted
Elosulfase alfa (3) Vimizim® BioMarin Deutschland Metabolic diseases Mucopolysaccharidoses (type IVA) n.d. discontinued Orphan
Repotrectinib (2) Augtyro® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Non-small cell lung cancer, ROS1-positive 585–1,620 100% additional benefit not proven
Repotrectinib (1) Augtyro® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Solid tumours, NTRK gene fusion, aged ≥ 12 years 390–770 100% additional benefit not proven
Ivacaftor / Tezacaftor / Elexacaftor (17) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, combination therapy with ivacaftor, ≥ 2 years, non-Class I mutation (a gating mutation and no F508del mutation) 100 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (16) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, combination therapy with ivacaftor, ≥ 2 years, non-Class I mutation (no F508del mutation and no gating mutation) 375 64% Hint for major additional benefit Orphan (turnover limit)
Serplulimab (1) Hetronifly® Accord Healthcare GmbH Oncological diseases Small cell lung cancer, in combination with carboplatin and etoposide, first-line treatment 3,210–7,719 100% Hint for non-quantifiable additional benefit Orphan
Bevacizumab (1) Lytenava® Outlook Therapeutics Limited Eye diseases Neovascular age-related macular degeneration 85,500–504,400 100% additional benefit not proven
Tiratricol (1) Emcitate® Rare Thyroid Therapeutics International AB Metabolic diseases Peripheral thyrotoxicosis associated with monocarboxylate transporter 8 deficiency (Allan-Herndon-Dudley syndrome), present from birth 40–110 100% Hint for non-quantifiable additional benefit Orphan
Trastuzumab deruxtecan (6) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases Breast cancer, HR+, HER2-low or -ultralow, following at least one course of endocrine therapy 1,615–6,200 100% Hint for minor additional benefit
Concizumab (2) Alhemo® Novo Nordisk GmbH Hematopoietic diseases Haemophilia B, aged ≥ 12 years, with factor IX inhibitors 2–12 57% Hint for considerable additional benefit
Concizumab (1) Alhemo® Novo Nordisk GmbH Hematopoietic diseases Haemophilia A, aged ≥ 12 years, with factor VIII inhibitors 80–125 100% additional benefit not proven
Pembrolizumab (40) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Malignant pleural mesothelioma, non-epithelioid, first-line treatment, in combination with pemetrexed and platinum-based chemotherapy 55–105 100% additional benefit not proven
Eplontersen (1) Wainzua® AstraZeneca GmbH Metabolic diseases Hereditary transthyretin amyloidosis with polyneuropathy (stage 1 or 2) 360 100% additional benefit not proven
Lisocabtagen maraleucel (3) Breyanzi® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Follicular lymphoma, following ≥ 2 prior treatments 370–840 100% additional benefit not proven
Pirtobrutinib (2) Jaypirca® Lilly Deutschland GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), relapsed or refractory, monotherapy 5,390–7,490 9% Hint for minor additional benefit
Sargramostim (1) Imreplys® Partner Therapeutics Ltd n.d. Sargramostim (1) n.d. exempted
Belzutifan (2) Welireg® MSD Sharp & Dohme GmbH Oncological diseases Von Hippel-Lindau syndrome (VHL)-associated tumours 80–970 100% additional benefit not proven
Belzutifan (1) Welireg® MSD Sharp & Dohme GmbH Oncological diseases Renal cell carcinoma, advanced, after ≥ 2 prior therapies 65–940 50% Hint for minor additional benefit
Acoramidis (1) Beyonttra® Bayer Vital GmbH Metabolic diseases Hereditary transthyretin amyloidosis in adult patients with cardiomyopathy 1,760–2,120 100% additional benefit not proven
Seladelpar (1) Seladelpar Gilead® Gilead Sciences GmbH Digestive system diseases Primary biliary cholangitis (combination with ursodeoxycholic acid) 6,000–13,000 100% Indication of minor additional benefit Orphan
Mirikizumab (2) Omvoh® Lilly Deutschland GmbH Digestive system diseases Crohn's disease, pre-treated 12,600–78,300 100% additional benefit not proven
Fosdenopterin (1) Nulibry® Sentynl Therapeutics Nervous system diseases Molybdenum cofactor deficiency type A 2 100% Hint for non-quantifiable additional benefit Orphan
Iptacopan (2) Fabhalta® Novartis Pharma GmbH Genitourinary system diseases Complement-3 glomerulopathy n.d. discontinued Orphan
Atogepant (1) Aquipta® AbbVie Deutschland GmbH & Co. KG Nervous system diseases Migraine prophylaxis 1,386,900–1,726,200 100% additional benefit not proven
Blinatumomab (8) Blincyto® Amgen GmbH Oncological diseases Acute lymphoblastic B-cell leukaemia, high-risk first relapse, Ph-, CD19+, ≥1 month and <1 year 1 100% Hint for non-quantifiable additional benefit Orphan
Blinatumomab (9) Blincyto® Amgen GmbH Oncological diseases Acute lymphoblastic B-cell leukaemia, Ph-, CD19+, newly diagnosed 160–270 100% Hint for considerable additional benefit Orphan
Blinatumomab (7) Blincyto® Amgen GmbH Oncological diseases Acute lymphoblastic B-cell leukaemia, relapsed/refractory, ≥ 1 month to < 1 year, after ≥ 2 prior therapies or after allogeneic stem cell transplantation 1 100% Hint for non-quantifiable additional benefit Orphan
Daratumumab (13) Darzalex® Janssen-Cilag GmbH Oncological diseases Systemic light-chain amyloidosis, first-line, combination with cyclophosphamide, bortezomib and dexamethasone 220–515 100% Hint for considerable additional benefit Orphan (turnover limit)
Fedratinib (2) Inrebic® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Myelofibrosis 640–1,710 100% Hint for non-quantifiable additional benefit Orphan
Garadacimab (1) Andembry® CSL Behring GmbH Other diseases Hereditary angioedema, prophylaxis, ≥ 12 years 140–440 100% Hint for considerable additional benefit
Nirsevimab (3) Beyfortus® Sanofi-Aventis Deutschland GmbH Infectious diseases Prevention of RSV infections, children during their 1st RSV season, which are not addressed in the therapeutic indication for RSV antibodies 541,000–555,000 100% Indication of considerable additional benefit
Setmelanotid (3) Imcivree® Rhythm Pharmaceuticals Inc. Metabolic diseases Obesity and control of hunger, POMC, PCSK1, LEPR deficiency or Bardet-Biedl syndrome, ≥ 2 to < 6 years of age 24–67 100% Hint for non-quantifiable additional benefit Orphan
Vibegron (1) Obgemsa® Pierre Fabre Pharma GmbH Genitourinary system diseases Overactive bladder 587,900–1,270,000 100% additional benefit not proven
Cabotegravir (2) Vocabria® ViiV Healthcare GmbH Infectious diseases HIV-1 infection, treatment-experienced adolescents, combination with rilpivirine, 12 to 17 years of age 65 100% additional benefit not proven
Nemolizumab (1) Nemluvio® Galderma Laboratorium GmbH Skin diseases Atopic dermatitis, ≥ 12 years 57,300–62,600 100% additional benefit not proven
Nemolizumab (2) Nemluvio® Galderma Laboratorium GmbH Skin diseases Prurigo nodularis 3,500–5,500 100% additional benefit not proven
Nintedanib (Ofev, 6) Ofev® Boehringer Ingelheim Pharma GmbH Respiratory system diseases Interstitial lung disease with systemic sclerosis, 6 to < 18 years of age 0–8 100% additional benefit not proven
Nintedanib (Ofev, 5) Ofev® Boehringer Ingelheim Pharma GmbH Respiratory system diseases Clinically significant progressive fibrosing interstitial lung disease, 6 to < 18 years of age 1–35 100% additional benefit not proven
Sarilumab (2) Kevzara® Sanofi-Aventis Deutschland GmbH Musculoskeletal system diseases Polyarticular juvenile idiopathic arthritis (pJIA), ≥ 2 years 1,370–1,480 100% additional benefit not proven
Sarilumab (3) Kevzara® Sanofi-Aventis Deutschland GmbH Musculoskeletal system diseases Polymyalgia rheumatica 10,300–14,200 100% additional benefit not proven
Sipavibart (1) Kavigale® AstraZeneca GmbH Infectious diseases COVID-19, pre-exposure prophylaxis, ≥ 12 years n.d. 100% additional benefit not proven
Dostarlimab (3) Jemperli® GlaxoSmithKline GmbH & Co. KG Oncological diseases Primarily advanced or recurrent endometrial cancer with pMMR, combination with carboplatin and paclitaxel 990–1,810 100% additional benefit not proven
Isatuximab (3) Sarclisa® Sanofi-Aventis Deutschland GmbH Oncological diseases Multiple myeloma, first-line, stem cell transplantation unsuitable, combination with bortezomib, lenalidomide and dexamethasone 3,450–3,680 100% Hint for minor additional benefit
Pirtobrutinib (1) Jaypirca® Lilly Deutschland GmbH Oncological diseases Mantle cell lymphoma, pre-treated patients 105–150 100% additional benefit not proven
Lazertinib (1) Lazcluze® Janssen-Cilag GmbH Oncological diseases Non-small cell lung cancer, EGFR exon 19 deletions or exon 21 substitution mutations (L858R), combination with amivantamab 1,250–3,025 100% Hint for minor additional benefit
Amivantamab (4) Rybrevant® Janssen-Cilag GmbH Oncological diseases Non-small cell lung cancer, EGFR exon 20 insertion mutation, first-line, combination with carboplatin and pemetrexed 70–215 100% additional benefit not proven
Amivantamab (2) Rybrevant® Janssen-Cilag GmbH Oncological diseases Non-small cell lung cancer, EGFR exon 19 deletions or exon 21 substitution mutations (L858R), combination with lazertinib 1,250–3,025 100% Hint for minor additional benefit
Amivantamab (3) Rybrevant® Janssen-Cilag GmbH Oncological diseases Non-small cell lung cancer, EGFR exon 19 deletions or exon 21 substitution mutations (L858R), pretreated, combination with carboplatin and pemetrexed 985–3,045 100% additional benefit not proven
Marstacimab (1) Hympavzi® Pfizer Pharma GmbH Hematopoietic diseases Severe haemophilia A, ≥ 12 years, without factor VIII inhibitors 1,900–2,000 100% additional benefit not proven
Marstacimab (2) Hympavzi® Pfizer Pharma GmbH Hematopoietic diseases Severe haemophilia B, ≥ 12 years, without factor IX inhibitors 300–310 100% additional benefit not proven
Natriumthiosulfat (1) Pedmarqsi® Norgine GmbH Other diseases Prevention of ototoxicity due to cisplatin chemotherapy, solid tumours, 1 month to < 18 years 38–228 16% Indication of non-quantifiable additional benefit
Erdnussprotein als entfettetes Pulver von Arachis hypogaea L., semen (Erdnüsse) (2) Palforzia® Stallergenes GmbH Other diseases Peanut allergy, ≥ 1 to < 4 years 9,960–22,700 100% Hint for non-quantifiable additional benefit
Exagamglogen autotemcel (2) Casgevy® Vertex Pharmaceuticals (Germany) GmbH Hematopoietic diseases Sickle cell disease with recurrent vaso-occlusive crises; ≥ 12 years; no HLA-matched related stem cell donation available 130–330 100% Hint for non-quantifiable additional benefit Orphan
Exagamglogen autotemcel (1) Casgevy® Vertex Pharmaceuticals (Germany) GmbH Hematopoietic diseases Β-thalassaemia, transfusion-dependent, ≥ 12 years, no HLA-compatible related stem cell donation available 20–150 100% Indication of non-quantifiable additional benefit Orphan
Osimertinib (7) Tagrisso® AstraZeneca GmbH Oncological diseases Non-small cell lung cancer, EGFR mutations, after platinum-based radiochemotherapy 160–290 100% additional benefit not proven
Bulevirtid (3) Hepcludex® Gilead Sciences GmbH Infectious diseases Hepatitis delta virus (HDV) infection, HDV RNA-positive n.d. discontinued Orphan
Bulevirtid (2) Hepcludex® Gilead Sciences GmbH Infectious diseases Hepatitis delta virus (HDV) infection, HDV RNA-positive, ≥ 3 to < 18 years, ≥ 10 kg bw n.d. discontinued Orphan
Eliglustat (2) Cerdelga® Sanofi-Aventis Deutschland GmbH Metabolic diseases Gaucher disease type 1, ≥ 6 to < 18 years, ≥ 15 kg bw 10–30 100% Hint for non-quantifiable additional benefit Orphan
Erdafitinib (1) Balversa® Janssen-Cilag GmbH Oncological diseases Urothelial carcinoma, FGFR3 alterations, pretreated with PD-(L)1 inhibitor 169–207 100% additional benefit not proven
Tislelizumab (6) Tevimbra® BeiGene Germany GmbH Oncological diseases Adenocarcinoma of the stomach or gastro-oesophageal junction, PD-L1 expression ≥ 5, HER2-, first-line, combination with platinum- and fluoropyrimidine-based chemotherapy 1,941–3,067 100% additional benefit not proven
Tislelizumab (4) Tevimbra® BeiGene Germany GmbH Oncological diseases Non-small cell lung cancer, after prior therapy 690–1,620 100% additional benefit not proven
Tislelizumab (2) Tevimbra® BeiGene Germany GmbH Oncological diseases Non-small cell lung cancer, non-squamous, PD-L1 expression ≥ 50%, first-line, combination with pemetrexed and platinum-containing chemotherapy 3,460–4,600 100% additional benefit not proven
Tislelizumab (3) Tevimbra® BeiGene Germany GmbH Oncological diseases Non-small cell lung cancer, squamous cell, first-line, combination with carboplatin and either paclitaxel or nab-paclitaxel 7,780–11,040 100% additional benefit not proven
Tislelizumab (5) Tevimbra® BeiGene Germany GmbH Oncological diseases Squamous cell carcinoma of the oesophagus, PD-L1 expression TAP score ≥ 5%, first-line, combination with platinum-based chemotherapy 1,530–2,050 100% additional benefit not proven
Tislelizumab (1) Tevimbra® BeiGene Germany GmbH Oncological diseases Squamous cell carcinoma of the oesophagus, after prior therapy 330–540 100% additional benefit not proven
Ribociclib (5) Kisqali® Novartis Pharma GmbH Oncological diseases Breast cancer, HR+, HER2-, early with high risk of recurrence, adjuvant therapy, combination with aromatase inhibitor 3,730–15,960 100% additional benefit not proven
Linzagolix (2) Yselty® Theramex Ireland Limited Genitourinary system diseases Endometriosis 8,100–13,900 100% additional benefit not proven
Mirvetuximab-Soravtansin (1) Elahere® AbbVie Deutschland GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, FRα-positive, platinum-resistant, after 1 to 3 prior therapies 0
630–1,300 		100% Indication of considerable additional benefit Orphan repealed
Benralizumab (2) Fasenra® AstraZeneca GmbH Musculoskeletal system diseases Eosinophilic granulomatosis with polyangiitis 90–1,360 100% additional benefit not proven
Daratumumab (12) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma, first-line, stem cell transplantation suitable, combination with bortezomib, lenalidomide and dexamethasone 1,750–1,910 100% additional benefit not proven Orphan (turnover limit)
Dupilumab (11) Dupixent® Sanofi-Aventis Deutschland GmbH Digestive system diseases Eosinophilic oesophagitis, ≥ 1 year to < 12 years 530–590 100% additional benefit not proven
Ciltacabtagen autoleucel (2) Carvykti® Janssen-Cilag GmbH Oncological diseases Multiple myeloma, after at least 1 prior therapy, refractory to lenalidomide 2,280–3,640 62% Hint for considerable additional benefit Orphan
Apremilast (3) Otezla® Amgen GmbH Skin diseases Moderate to severe plaque psoriasis; 6 to < 18 years of age 360–430 100% additional benefit not proven
Ceftazidim / Avibactam (2) Zavicefta® Pfizer Pharma GmbH Infectious diseases Bacterial infections, several therapeutic indications, from birth to < 3 years 10–27 100% additional benefit considered proven
Pembrolizumab (38) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Cervical carcinoma (stage III to IVA), first-line, combination with radiochemotherapy 750 100% Indication of non-quantifiable additional benefit
Pembrolizumab (39) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Endometrial carcinoma, first-line, combination with carboplatin and paclitaxel 1,370–3,330 100% additional benefit not proven
Artesunat (1) Artesunate Amivas® Amivas Ireland Ltd Infectious diseases Severe malaria, from birth 87 100% non-quantifiable additional benefit Orphan
Epcoritamab (3) Tepkinly® AbbVie Deutschland GmbH & Co. KG Oncological diseases Diffuse large B-cell lymphoma (DLBCL), after ≥ 2 prior therapies 920–1,940 100% additional benefit not proven
Meropenem / Vaborbactam (1) Vaborem® Berlin-Chemie AG Infectious diseases Acterial infections, several therapeutic indications 1,294–1,405 100% additional benefit considered proven
Zolbetuximab (1) Vyloy® Astellas Pharma GmbH Oncological diseases Adenocarcinoma of the stomach or gastro-oesophageal junction, HER2-negative, first-line, combination with fluoropyrimidine- and platinum-containing chemotherapy 250–1,310 100% Indication of minor additional benefit Orphan
Delgocitinib (1) Anzupgo® LEO Pharma GmbH Skin diseases Moderate to severe chronic hand eczema 160,000–200,000 100% additional benefit not proven
Elafibranor (1) Iqirvo® Ipsen Pharma GmbH Digestive system diseases Primary biliary cholangitis (combination with ursodeoxycholic acid) 6,000–13,000 100% Hint for minor additional benefit Orphan
Enfortumab Vedotin (2) Padcev® Astellas Pharma Europe B.V Oncological diseases Urothelial carcinoma, non-resectable or metastatic, first-line, suitable for platinum-containing chemotherapy, combination with pembrolizumab 920–2,280 45% Indication of considerable additional benefit
Pembrolizumab (36) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Urothelial carcinoma, non-resectable or metastatic, first-line, combination with enfortumab vedotin 1,050–2,601 39% Indication of considerable additional benefit
Capivasertib (1) Truqap® AstraZeneca GmbH Oncological diseases Breast cancer, ER+, HER2-, PIK3CA/AKT1/PTEN alteration(s), after prior therapy, combination with fulvestrant 491–26,745 70% Indication of considerable additional benefit
Encorafenib (3) Braftovi® Pierre Fabre Pharma GmbH Oncological diseases Non-small cell lung cancer, advanced, BRAF V600E mutation, combination with binimetinib 122–476 100% additional benefit not proven
Pembrolizumab (37) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Breast cancer, triple-negative, high risk of recurrence, neoadjuvant and adjuvant therapy, monotherapy or combination with chemotherapy 2,440–2,610 50% Indication of minor additional benefit
Binimetinib (2) Mektovi® Pierre Fabre Pharma GmbH Oncological diseases Non-small cell lung cancer, advanced, BRAF V600E mutation, combination with encorafenib 122–476 100% additional benefit not proven
Atezolizumab (12) Tecentriq® Roche Pharma AG Oncological diseases Non-small cell lung cancer, PD-L1 expression ≥ 50%, adjuvant therapy after resection and chemotherapy 700–890 100% Hint for considerable additional benefit
Atezolizumab (11) Tecentriq® Roche Pharma AG Oncological diseases Non-small cell lung cancer, first-line 1,900–7,570 71% Indication of minor additional benefit
Polihexanid (1) Akantior® SIFI S.p.A. Eye diseases Acanthamoebic keratitis; ≥ 12 years of age 70–410 100% Hint for non-quantifiable additional benefit Orphan
Isavuconazol (3) Cresemba® Pfizer Pharma GmbH Infectious diseases Mucormycosis, ≥ 1 to ≤ 17 years 8 100% Hint for non-quantifiable additional benefit Orphan
Isavuconazol (2) Cresemba® Pfizer Pharma GmbH Infectious diseases Aspergillosis, ≥ 1 to ≤ 17 years 40–215 100% Hint for non-quantifiable additional benefit Orphan
Epcoritamab (2) Tepkinly® AbbVie Deutschland GmbH & Co. KG Oncological diseases Follicular lymphoma, after ≥ 2 prior therapies 370–840 100% additional benefit not proven
Crovalimab (1) Piasky® Roche Pharma AG Hematopoietic diseases Paroxysmal nocturnal haemoglobinuria, ≥ 12 years, ≥ 40 kg 260–749 100% additional benefit not proven
Sotatercept (1) Winrevair® MSD Sharp & Dohme GmbH Cardiovascular diseases Pulmonary arterial hypertension 580–7,850 100% Hint for minor additional benefit Orphan
Linzagolix (1) Yselty® Theramex Ireland Limited Genitourinary system diseases Uterine fibroid 20,160–100,800 100% additional benefit not proven
Faricimab (3) Vabysmo® Roche Pharma AG Eye diseases Macular oedema due to retinal vein occlusion 59,200–96,400 100% additional benefit not proven
Insulin icodec (1) Awiqli® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 2 855,000–1,184,000 100% additional benefit not proven
Insulin icodec (2) Awiqli® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 1 161,750 100% additional benefit not proven
Risankizumab (4) Skyrizi® AbbVie Deutschland GmbH & Co. KG Digestive system diseases Ulcerative colitis, pre-treated 29,100 100% additional benefit not proven
Aztreonam / Avibactam (1) Emblaveo® Pfizer Pharma GmbH Infectious diseases Bacterial infections, several therapeutic indications 2,600–6,600 100% additional benefit considered proven
Olaparib (12) Lynparza® AstraZeneca GmbH Oncological diseases Primarily advanced or recurrent endometrial cancer with pMMR, combination with durvalumab, maintenance therapy 780–1,430 50% Indication of considerable additional benefit
Durvalumab (7) Imfinzi® AstraZeneca GmbH Oncological diseases Primarily advanced or relapsed endometrial cancer, first-line therapy, combination with carboplatin and paclitaxel; maintenance therapy 380–1,520 100% additional benefit not proven
Durvalumab (8) Imfinzi® AstraZeneca GmbH Oncological diseases Primarily advanced or recurrent endometrial cancer, combination with carboplatin and paclitaxel; maintenance therapy, combination with olaparib 990–1,810 50% Indication of considerable additional benefit
RADAMTS13 (1) Adzynma® Takeda GmbH Oncological diseases ADAMTS13 deficiency in congenital thrombotic thrombocytopenic purpura (cTTP) 120–180 100% Hint for non-quantifiable additional benefit Orphan
Nirsevimab (2) Beyfortus® Sanofi-Aventis Deutschland GmbH Infectious diseases Secondary prophylaxis of RSV infections, children during their 2nd RSV season, ≥ 24 months of life 9,535 100% additional benefit not proven
Sparsentan (1) Filspari® Vifor Pharma Deutschland GmbH Genitourinary system diseases Immunoglobulin A - Nephropathy, primary 900–13,000 100% Hint for minor additional benefit Orphan
Osimertinib (6) Tagrisso® AstraZeneca GmbH Oncological diseases Non-small cell lung cancer, first-line, combination with pemetrexed and platinum-containing chemotherapy 840–2,720 100% additional benefit not proven
Maralixibat (2) Livmarli® Mirum Pharmaceuticals International B.V. Digestive system diseases Progressive familial intrahepatic cholestasis (PFIC), ≥ 3 months 80–180 100% Hint for non-quantifiable additional benefit Orphan
Entrectinib (3) Rozlytrek® Roche Pharma AG Oncological diseases Solid tumors, neurotrophic tyrosine receptor kinase (NTRK) gene fusion, histology-independent, > 1 month to < 12 years 3 100% additional benefit not proven
Dupilumab (10) Dupixent® Sanofi-Aventis Deutschland GmbH Respiratory system diseases COPD 9,370 71% Indication of minor additional benefit
Alectinib (3) Alecensa® Roche Pharma AG Oncological diseases Non-small cell lung cancer, ALK+, high risk of recurrence, adjuvant therapy 330–452 50% Hint for major additional benefit
Gozetotid (1) Locametz® Novartis Pharma GmbH Oncological diseases Detection of PSMA-positive prostate cancer (mCRPC), PSMA-targeted therapy 1,860–2,770 84% Indication of considerable additional benefit
Fruquintinib (1) Fruzaqla® Takeda GmbH Oncological diseases Colorectal carcinoma, previously treated patients 645–2,180 100% Hint for minor additional benefit
Nivolumab (28) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Urothelial carcinoma, first-line, combination with cisplatin and gemcitabine 435–617 100% additional benefit not proven
Axicabtagen-Ciloleucel (7) Yescarta® Gilead Sciences GmbH Oncological diseases Diffuse large B-cell lymphoma, highly malignant B-cell lymphoma, after 1 prior therapy, relapse within 12 months or refractory 800–1,130 100% Hint for minor additional benefit Orphan (turnover limit)
Osimertinib (5) Tagrisso® AstraZeneca GmbH Oncological diseases Non-small cell lung cancer, EGFR mutations, adjuvant therapy 640–930 50% Hint for major additional benefit
Iptacopan (1) Fabhalta® Novartis Pharma GmbH Hematopoietic diseases Paroxysmal nocturnal hemoglobinuria 0
290–945 		100% Hint for non-quantifiable additional benefit Orphan repealed
Tofersen (1) Qalsody® Biogen GmbH Nervous system diseases Amyotrophic lateral sclerosis (ALS) 90–170 100% Hint for non-quantifiable additional benefit Orphan
Cefepim / Enmetazobactam (1) Exblifep® Advanz Pharma Germany GmbH Infectious diseases Bacterial infections, several areas of application 1,000–2,600 100% additional benefit considered proven
Pegcetacoplan (2) Aspaveli® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Paroxysmal nocturnal hemoglobinuria, untreated patients 100–425 100% Hint for non-quantifiable additional benefit Orphan
Vadadustat (1) Vafseo® MEDICE Arzneimittel Pütter GmbH & Co. KG Hematopoietic diseases Symptomatic anemia in chronic kidney disease (CKD) 60,800–71,400 100% additional benefit not proven
Futibatinib (1) Lytgobi® Taiho Pharma Netherlands Oncological diseases Cholangiocarcinoma, with FGFR2 fusion or FGFR2 rearrangement, after at least 1 prior therapy 27–229 100% additional benefit not proven
Danicopan (1) Voydeya® Alexion Pharma Germany GmbH Hematopoietic diseases Paroxysmal hemoglobinuria in residual hemolytic anemia, add-on therapy to ravulizumab or eculizumab) 70–350 100% Hint for non-quantifiable additional benefit Orphan
Bimekizumab (5) Bimzelx® UCB Pharma GmbH Skin diseases Hidradenitis suppurativa (acne inversa)) 2,800–6,400 100% additional benefit not proven
Selpercatinib (7) Retsevmo® Lilly Deutschland GmbH Oncological diseases Solid tumors, RET-Fusion+ 59–159 100% additional benefit not proven
Selpercatinib (6) Retsevmo® Lilly Deutschland GmbH Oncological diseases Thyroid carcinoma, RET fusion+, refractory to radioiodine, first-line or after systemic prior therapy, ≥ 12 years of age 6–36 100% additional benefit not proven
Melatonin (2) Slenyto® InfectoPharm Arzneimittel und Consilium GmbH Nervous system diseases Sleep disorders in neurogenetic diseases; ≥ 2 to ≤ 18 years of age n.d. discontinued
Dabrafenib (Finlee, 1) Finlee® Novartis Pharma GmbH Oncological diseases Malignant glioma, BRAF V600E mutation, ≥ 1 year, low-grade (LGG) first-line/higher-grade (HGG) after at least 1 prior therapy; combination with trametinib 7–115 41% Hint for considerable additional benefit Orphan
Trametinib (Spexotras, 1) Spexotras® Novartis Pharma GmbH Oncological diseases Malignant glioma, BRAF V600E mutation, ≥ 1 year, low-grade (LGG) first-line/higher-grade (HGG) after at least 1 prior therapy; combination with dabrafenib 7–115 41% Hint for considerable additional benefit Orphan
Luspatercept (6) Reblozyl® Bristol-Myers Squibb GmbH & Co. KGaA Hematopoietic diseases Myelodysplastic syndromes with transfusion-dependent anemia, not pretreated, and without ring sideroblasts, pretreated 4,960–7,080 80% Hint for minor additional benefit Orphan (turnover limit)
Pembrolizumab (35) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Non-small cell lung cancer, high risk of recurrence, neoadjuvant and adjuvant therapy, monotherapy or combination with platinum-based chemotherapy 5,090–6,780 100% additional benefit not proven
Pembrolizumab (34) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Non-small cell lung cancer, adjuvant therapy with high risk of recurrence after resection, after prior chemotherapy 2,690–3,200 100% additional benefit not proven
Abrocitinib (2) Cibinqo® Pfizer Pharma GmbH Skin diseases Atopic dermatitis, ≥ 12 to < 18 years 5,300–10,600 100% additional benefit not proven
Efbemalenograstim alfa (1) Ryzneuta® Evive Biotech Oncological diseases Febrile neutropenia due to chemotherapy n.d. 100% additional benefit not proven
Efanesoctocog alfa (1) Altuvoct® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Hemophilia A n.d. 100% additional benefit not proven Orphan
Etrasimod (1) Velsipity® Pfizer Pharma GmbH Digestive system diseases Ulcerative colitis, ≥ 16 years 29,100 100% additional benefit not proven
Abaloparatid (1) Eladynos® Theramex Germany GmbH Musculoskeletal system diseases Osteoporosis, postmenopausal women 484,000 100% additional benefit not proven
Efgartigimod alfa (2) Vyvgart® Argenx Germany GmbH Nervous system diseases Myasthenia gravis, AChR antibodies+ 6,300–19,000 100% additional benefit not proven Orphan (turnover limit)
Gadopiclenol (1) Elucirem® Guerbet GmbH Other diseases Contrast-enhanced magnetic resonance imaging, ≥ 2 years 2,900,000–3,300,000 100% additional benefit not proven
Idecabtagen vicleucel (3) Abecma® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Multiple myeloma, at least 2 prior therapies 4,900–5,250 100% additional benefit not proven Orphan (turnover limit)
Omaveloxolon (1) Skyclarys® Biogen GmbH Nervous system diseases Friedreich's ataxia, ≥ 16 years 0
970 		100% Hint for non-quantifiable additional benefit Orphan repealed
Zilucoplan (1) Zilbrysq® UCB Pharma GmbH Nervous system diseases Myasthenia gravis, AChR antibody+ 6,300–19,000 100% additional benefit not proven
Rozanolixizumab (1) Rystiggo® UCB Pharma GmbH Nervous system diseases Myasthenia gravis, AChR antibodies+, MuSK antibodies+ 6,470–19,300 98% Hint for considerable additional benefit Orphan
Enalapril (1) Aqumeldi® Proveca Pharma Limited Cardiovascular diseases Heart failure, < 18 years 0
3,070 		100% additional benefit not proven repealed
Decitabin / Cedazuridin (1) Inaqovi® Otsuka Pharma GmbH Oncological diseases Acute myeloid leukaemia, first line 560–840 100% additional benefit not proven
Daridorexant (2) Quviviq® Idorsia Pharmaceuticals Germany GmbH Nervous system diseases Sleep disorders 8,300–218,500 100% additional benefit not proven
Nirsevimab (1) Beyfortus® Sanofi-Aventis Deutschland GmbH Infectious diseases Secondary prophylaxis of RSV infections, children during their 1st RSV season 52,450–66,450 100% additional benefit not proven
Momelotinib (1) Omjjara® GlaxoSmithKline GmbH & Co. KG Oncological diseases Myelofibrosis 0
210–1,160 		50% Hint for minor additional benefit Orphan repealed
Talazoparib (2) Talzenna® Pfizer Pharma GmbH Oncological diseases Prostate carcinoma, metastasised, castration-resistant, in combination with enzalutamide) 9,400–12,200 75% additional benefit not proven
Rezafungin (1) Rezzayo® Mundipharma Infectious diseases Invasive candida infections 31,800–34,600 100% Hint for non-quantifiable additional benefit Orphan
Patiromer (2) Veltassa® Fresenius Medical Care Nephrologica Deutschland GmbH Metabolic diseases Hyperkalaemia, ≥ 12 to < 18 years 370–790 100% additional benefit not proven
Fezolinetant (1) Veoza® Astellas Pharma GmbH Other diseases Severe vasomotor symptoms (VMS) associated with the menopause 2,589,650–3,015,900 80% Hint for minor additional benefit
Ublituximab (1) Briumvi® Neuraxpharm Arzneimittel GmbH Nervous system diseases Relapsing-remitting multiple sclerosis 70,300–274,900 63% Indication of minor additional benefit
Quizartinib (1) Vanflyta® Daiichi Sankyo Deutschland GmbH Oncological diseases Acute myeloid leukaemia (AML) 260–820 100% additional benefit not proven
Brentuximab Vedotin (13) Adcetris® Takeda GmbH Oncological diseases Systemic anaplastic large cell lymphoma; first-line; combination with cyclophosphamide, doxorubicin and prednisone n.d. discontinued Orphan
Brentuximab Vedotin (8) Adcetris® Takeda GmbH Oncological diseases Hodgkin's lymphoma, CD30+, pre-treated n.d. discontinued Orphan
Brentuximab Vedotin (9) Adcetris® Takeda GmbH Oncological diseases Systemic anaplastic large cell lymphoma (sALCL) , relapsed, refractory n.d. discontinued Orphan
Brentuximab Vedotin (10) Adcetris® Takeda GmbH Oncological diseases Hodgkin's lymphoma, CD30+, increased risk of recurrence or progression after transplantation n.d. discontinued Orphan
Brentuximab Vedotin (11) Adcetris® Takeda GmbH Oncological diseases Cutaneous T-cell lymphoma, CD30+, pre-treated n.d. discontinued Orphan
Brentuximab Vedotin (12) Adcetris® Takeda GmbH Oncological diseases Hodgkin's lymphoma, CD30+, first-line, stage III + IV, in combination with doxorubicin, vinblastine and dacarbazine n.d. discontinued Orphan
Brentuximab Vedotin (7) Adcetris® Takeda GmbH Oncological diseases Hodgkin-Lymphoma (CD30+, first-line, stadium III, in combination with Doxorubicin, Vinblastin and Dacarbazin) n.d. discontinued Orphan
Pegzilarginase (1) Loargys® Immedica Pharma Germany GmbH Metabolic diseases Hyperargininemia (ARG1-D), ≥ 2 years 50 100% Hint for non-quantifiable additional benefit Orphan
Elranatamab (1) Elrexfio® Pfizer Pharma GmbH Oncological diseases Multiple myeloma, relapsed and refractory, after at least 3 previous therapies 1,250–1,340 100% additional benefit not proven
Vamorolon (1) Agamree® Santhera Pharmaceuticals GmbH Musculoskeletal system diseases Duchenne muscular dystrophy, ≥ 4 years 740–3,670 100% Hint for non-quantifiable additional benefit Orphan
Evinacumab (1) Evkeeza® Ultragenyx Germany GmbH Metabolic diseases Homozygous familial hypercholesterolemia, ≥ 12 years of age 73–80 100% additional benefit not proven
Evinacumab (2) Evkeeza® Ultragenyx Germany GmbH Metabolic diseases Homozygous familial hypercholesterolemia, ≥ 5 to < 12 years of age 5–6 100% additional benefit not proven
Pembrolizumab (33) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Biliary tumours, first-line, combination with gemcitabine and cisplatin 1,480–2,180 100% Indication of minor additional benefit
Pembrolizumab (31) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Adenocarcinoma of the stomach or gastroesophageal junction, PD-L1 expression ≥ 1, HER2+, first-line, combination with trastuzumab, fluoropyrimidine- and platinum-based chemotherapy 70–710 100% additional benefit not proven
Pembrolizumab (32) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Adenocarcinoma of the stomach or gastroesophageal junction, PD-L1 expression ≥ 1, HER2, first-line, combination with fluoropyrimidine- and platinum-based chemotherapy 285–2,613 100% additional benefit not proven
Palopegteriparatid (1) Yorvipath® Ascendis Pharma Endocrinology GmbH Metabolic diseases Chronic hypoparathyroidism, parathyroid hormone (PTH) substitution therapy 18,300–20,800 100% additional benefit not proven Orphan
Dostarlimab (2) Jemperli® GlaxoSmithKline GmbH & Co. KG Oncological diseases Primary advanced or recurrent endometrial carcinoma with dMMR/ MSI-H, combination with carboplatin and paclitaxel 790–1,520 50% Indication of major additional benefit
Empagliflozin (6) Jardiance® Boehringer Ingelheim Pharma GmbH Metabolic diseases Diabetes Mellitus Type 2, ≥ 10 years 300–385 100% additional benefit not proven
Avapritinib (3) Ayvakyt® Blueprint Medicines GmbH Oncological diseases Indolent systemic mastocytosis (ISM) 0
715–1,000 		100% Indication of minor additional benefit Orphan repealed
Polatuzumab Vedotin (4) Polivy® Roche Pharma AG Oncological diseases Diffuse large B-cell lymphoma, combination with rituximab, cyclophosphamide, doxorubicin and prednisone (R-CHP) 5,640–6,270 100% additional benefit not proven Orphan (turnover limit)
Polatuzumab Vedotin (3) Polivy® Roche Pharma AG Oncological diseases Diffuse large B-cell lymphoma, combination with bendamustine and rituximab 2,550–3,120 100% additional benefit not proven Orphan (turnover limit)
Zanubrutinib (5) Brukinsa® BeiGene Germany GmbH Oncological diseases Follicular lymphoma, after ≥ 2 prior therapies, combination with obinutuzumab 370–840 100% additional benefit not proven
Rucaparib (4) Rubraca® Pharmaand Deutschland GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, maintenance therapy after first-line therapy 3,250–3,590 100% additional benefit not proven
Durvalumab (6) Imfinzi® AstraZeneca GmbH Oncological diseases Hepatocellular carcinoma, first-line, monotherapy 1,900–5,470 100% additional benefit not proven
Alirocumab (3) Praluent® Sanofi-Aventis Deutschland GmbH Metabolic diseases Hypercholesterolaemia, ≥ 8 years to 17 years 958–1,178 100% additional benefit not proven
Lebrikizumab (1) Ebglyss® Almirall Hermal GmbH Skin diseases Atopic dermatitis, ≥ 12 years 57,300–62,600 100% additional benefit not proven
Letermovir (3) Prevymis® MSD Sharp & Dohme GmbH Infectious diseases CMV reactivation/disease, prophylaxis after stem cell transplantation 1,400–1,800 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Letermovir (2) Prevymis® MSD Sharp & Dohme GmbH Infectious diseases CMV disease, prophylaxis after kidney transplantation 320 100% additional benefit not proven Orphan (turnover limit)
Lanadelumab (3) Takhzyro® Takeda GmbH Other diseases Hereditary angioedema, prophylaxis, 2 to < 12 years 1–30 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (11) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, combination treatment with ivacaftor, from 2 to ≤ 5 years of age (heterozygous for F508del and MF mutation) 160 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (12) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, combination treatment with ivacaftor, 2 to ≤ 5 years (homozygous for F508del mutation) 250 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (13) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, F508del mutation, heterozygous and gating mutation, ≥ 2 to ≤ 5 years, combination with ivacaftor 13 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (14) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, F508del mutation, heterozygous and RF mutation, ≥ 2 to ≤ 5 years, combination with ivacaftor 20 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (15) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, F508del mutation, heterozygous and other or unknown mutation, ≥ 2 to ≤ 5 years, combination with ivacaftor 33 100% additional benefit not proven Orphan (turnover limit)
Cannabidiol (6) Epidyolex® Jazz Pharmaceuticals Germany GmbH Nervous system diseases Dravet syndrome, ≥ 2 years, combination with clobazam 0
500–2,900 		100% additional benefit not proven Orphan (turnover limit) repealed
Cannabidiol (7) Epidyolex® Jazz Pharmaceuticals Germany GmbH Nervous system diseases Lennox-Gastaut syndrome, ≥ 2 years, combination with clobazam 1,700–22,700 100% additional benefit not proven Orphan (turnover limit)
Cannabidiol (8) Epidyolex® Jazz Pharmaceuticals Germany GmbH Other diseases Seizures associated with tuberous sclerosis, ≥ 2 years 200–2,700 100% additional benefit not proven Orphan (turnover limit)
Tebentafusp (2) Kimmtrak® Immunocore Ireland Ltd. Oncological diseases Uveal melanoma, HLA-A*02:01-positive 100–130 100% Hint for considerable additional benefit Orphan (turnover limit)
Patisiran (2) Onpattro® Alnylam Germany GmbH Metabolic diseases Hereditary transthyretin amyloidosis with polyneuropathy (stage 1 or 2) 360 100% Indication of less benefit Orphan (turnover limit)
Daratumumab (11) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma, first-line, stem cell transplantation unsuitable, combination with bortezomib, melphalan and prednisone 3,450–2,680 100% Indication of considerable additional benefit Orphan (turnover limit)
Relugolix / Estradiol / Norethisteronacetat (2) Ryeqo® Gedeon Richter Pharma GmbH Allgemeine Genitourinary system diseases Endometriosis, after medical or surgical treatment 8,200–13,900 100% additional benefit not proven
Vosoritid (3) Voxzogo® BioMarin International Limited Other diseases Achondroplasia, ≥ 4 months to < 2 years 35–49 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Trastuzumab deruxtecan (5) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases Non-small cell lung cancer, HER2(ERBB2) mutation, pre-treated 75–219 100% additional benefit not proven
Idecabtagen vicleucel (2) Abecma® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Multiple myeloma, at least 3 previous therapies n.d. discontinued Orphan (turnover limit)
Tirzepatid (1) Mounjaro® Lilly Deutschland GmbH Metabolic diseases Diabetes mellitus type 2 1,461,000–2,021,000 23% Hint for minor additional benefit
Niraparib / Abirateronacetat (1) Akeega® Janssen-Cilag GmbH Oncological diseases Prostate carcinoma, metastastic, castration resistent, BRCA1/2-Mutation, Chemotherapy not indicated, combination with prednis(ol)one 1,030–2,200 50% Hint for considerable additional benefit
Midostaurin (3) Rydapt® Novartis Pharma AG Oncological diseases Systemic Mastocytosis 300–400 100% additional benefit not proven Orphan (turnover limit)
Midostaurin (2) Rydapt® Novartis Pharma AG Oncological diseases Acute myeloic Leukemia, FLT3-Mutation 380–1,040 100% additional benefit not proven Orphan (turnover limit)
Baricitinib (3) Olumiant® Lilly Deutschland GmbH Musculoskeletal system diseases Polyarticular juvenile idiopathic Arthritis, RF+ or RF− polyarticular and expanded oligoarticular, ≥ 2 years 1,370–1,410 100% additional benefit not proven
Baricitinib (6) Olumiant® Lilly Deutschland GmbH Skin diseases Juvenile Psoriatic Arthritis, ≥ 2 years 140–240 100% additional benefit not proven
Baricitinib (5) Olumiant® Lilly Deutschland GmbH Musculoskeletal system diseases Enthesitis-associated arthritis, ≥ 2 years 330–390 100% additional benefit not proven
Baricitinib (4) Olumiant® Lilly Deutschland GmbH Skin diseases Atopic dermatitis, ≥ 2 until < 17 years 17,700–28,600 100% additional benefit not proven
Elacestrant (1) Orserdu® Stemline Therapeutics B.V. Oncological diseases Breast carcinoma, ER+, HER2-, with ESR1 mutation, after min. 1 previous therapy 1,527–16,070 39% Indication of considerable additional benefit
Somapacitan (1) Sogroya® Novo Nordisk Pharma GmbH Metabolic diseases Growth disturbance due to growth hormone deficiency, ≥ 3 years 9,150–11,600 100% Hint for non-quantifiable additional benefit Orphan
Brolucizumab (3) Beovu® Novartis Pharma GmbH Eye diseases Neovascular age-related macular degeneration 85,200–681,400 100% additional benefit not proven
Melphalanflufenamid (2) Pepaxti® Oncopeptides AB Oncological diseases Multiple myeloma, refractory, after 2 prior therapies, combination with dexamethasone n.d. discontinued
Epcoritamab (1) Tepkinly® Abbvie Deutschland GmbH & Co. KG Oncological diseases Diffuse large B-cell lymphoma (DLBCL), after ≥ 2 prior therapies) 0
1,050–1,900 		100% Hint for non-quantifiable additional benefit Orphan repealed
Sirolimus (1) Hyftor® Plusultra pharma GmbH Skin diseases Facial angiofibroma in tuberous sclerosis, ≥ 6 years. 1,500–5,000 100% Hint for non-quantifiable additional benefit Orphan
Pegunigalsidase alfa (1) Elfabrio® Chiesi GmbH Metabolic diseases Morbus Fabry 60–1,260 100% additional benefit not proven
Nivolumab (27) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma (stage IIB or IIC), adjuvant therapy, ≥ 12 years, monotherapy). 1,620–2,310 100% additional benefit not proven
Talquetamab (1) Talvey® Janssen-Cilag GmbH Oncological diseases Multiple myeloma, at least 3 prior therapies 1,210–1,310 100% Hint for non-quantifiable additional benefit Orphan
Risdiplam (2) Evrysdi® Roche Pharma AG Nervous system diseases Spinal muscular atrophy, < 2 months 56–95 100% additional benefit not proven
Lonapegsomatropin (1) Skytrofa® Ascendis Pharma Endocrinology GmbH Metabolic diseases Growth disturbance due to growth hormone deficiency, ≥ 3 to < 18 years. 5,710–6,550 100% Hint for non-quantifiable additional benefit Orphan
Teclistamab (1) Tecvayli® Janssen-Cilag GmbH Oncological diseases Multiple myeloma, at least 3 prior therapies 1,210–1,310 100% additional benefit not proven
Vosoritid (2) Voxzogo® BioMarin International Limited Other diseases Achondroplasia, ≥ 2 years 330–460 100% Indication of non-quantifiable additional benefit Orphan (turnover limit)
Tisagenlecleucel (6) Kymriah® Novartis Pharma GmbH Oncological diseases B-cell acute lymphoblastic leukemia, relapsed/refractory, 0 ≤ 25 years). 40–90 100% Hint for non-quantifiable additional benefit Orphan
Tisagenlecleucel (7) Kymriah® Novartis Pharma GmbH Oncological diseases Diffuse large B-cell lymphoma, relapsed or refractory, ≥ 2 prior therapies) 530–1,200 100% Hint for non-quantifiable additional benefit Orphan
Nonacog beta pegol (2) Refixia® Novo Nordisk Pharma GmbH Hematopoietic diseases Hemophilia B, < 12 years 100–110 100% additional benefit not proven
Sacituzumab govitecan (2) Trodelvy® Gilead Sciences GmbH Oncological diseases Breast carcinoma, HR+, HER2-, at least 3 prior therapies 2,480–8,240 100% Indication of considerable additional benefit
Migalastat (3) Galafold® Amicus Therapeutics GmbH Metabolic diseases Fabry disease, ≥ 12 years 20–460 100% additional benefit not proven Orphan (turnover limit)
Trifluridin / Tipiracil (4) Lonsurf® Servier Deutschland GmbH Oncological diseases Colorectal cancer, after 2 prior therapies, combination with bevacizumab) 3,530–6,230 100% Hint for considerable additional benefit
Bedaquilin (5) Sirturo® Janssen-Cilag GmbH Infectious diseases Multidrug-resistant pulmonary tuberculosis 70–100 100% Hint for considerable additional benefit Orphan
Nivolumab (26) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Non-small cell lung cancer, PD-L1 expression ≥ 1 %, neoadjuvant therapy, combination with platinum-based chemotherapy 110–990 100% Hint for non-quantifiable additional benefit
Glofitamab (1) Columvi® Roche Pharma AG Oncological diseases B-cell lymphoma, diffuse large cell (DLBCL) 0
1,360–1,900 		100% Hint for non-quantifiable additional benefit Orphan repealed
Mavacamten (1) Camzyos® Bristol-Myers Squibb GmbH & Co. KGaA Cardiovascular diseases Symptomatic hypertrophic obstructive cardiomyopathy (NYHA Klasse II–III) 18,900–19,500 100% Hint for considerable additional benefit
Eftrenonacog alfa (2) Alprolix® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Hemophilia B 560–720 100% additional benefit not proven Orphan (turnover limit)
Empagliflozin (5) Jardiance® Boehringer Ingelheim Pharma GmbH Genitourinary system diseases Chronic renal insufficiency 2,259,300–2,478,100 100% additional benefit not proven
Dalbavancin (2) Xydalba® Advanz Pharma Germany GmbH Infectious diseases Acute bacterial skin and soft tissue infections (ABSSSI), ≥ 3 months 190–9,800 100% additional benefit considered proven
Cipaglucosidase alfa (1) Pombiliti® Amicus Therapeutics GmbH Metabolic diseases Pompe's disease, combination with miglustat 170–1,760 100% Hint for minor additional benefit
Lumacaftor / Ivacaftor (5) Orkambi® Vertex Pharmaceuticals Metabolic diseases Cystic fibrosis, homozygous F508del mutation in CFTR gene, 1 to < 2 years) 50 100% Hint for non-quantifiable additional benefit
Ivosidenib (2) Tibsovo® Servier Deutschland GmbH Oncological diseases Cholangiocarcinoma with IDH1-R132 mutation, after at least 1 prior therapy 80–160 100% Hint for non-quantifiable additional benefit Orphan
Ivosidenib (1) Tibsovo® Servier Deutschland GmbH Oncological diseases Acute myeloid leukemia with IDH1-R132 mutation, first-line, combination with azacitidine 45–125 100% Indication of major additional benefit Orphan
Mirikizumab (1) Omvoh® Lilly Deutschland GmbH Digestive system diseases Ulcerative colitis, pre-treated 5,300–25,000 100% additional benefit not proven
Dimethylfumarat (2) Tecfidera® Biogen GmbH Nervous system diseases Relapsing-remitting multiple sclerosis (MS) (new indication: children and adolescents aged 13 years and older)) 350–1,200 100% additional benefit not proven
Nivolumab (24) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, adolescents ≥ 12 to 18 years, monotherapy or combination with ipilimumab). 2–4 100% additional benefit not proven
Nivolumab (25) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, adjuvant therapy, adolescents ≥ 12 to 18 years, monotherapy 1–4 100% additional benefit not proven
Axicabtagen-Ciloleucel (4) Yescarta® Gilead Sciences GmbH Oncological diseases Follicular lymphoma, after ≥ 3 prior therapies 60–270 100% additional benefit not proven Orphan (turnover limit)
Axicabtagen-Ciloleucel (6) Yescarta® Gilead Sciences GmbH Oncological diseases Diffuse large B-cell lymphoma and primary mediastinal large B-cell lymphoma, after at least 2 prior therapies 680–1,200 100% additional benefit not proven Orphan (turnover limit)
Axicabtagen-Ciloleucel (5) Yescarta® Gilead Sciences GmbH Oncological diseases Diffuse large B-cell lymphoma, highly malignant B-cell lymphoma, after 1 prior therapy, relapse within 12 months or refractory 800–1,130
1,600–2,260 		50% Hint for non-quantifiable additional benefit Orphan (turnover limit) repealed subpopulations
Riociguat (4) Adempas® MSD SHARP & DOHME GMBH Cardiovascular diseases Pulmonary arterial hypertension, < 18 years of age 5–35 100% additional benefit not proven
Bimekizumab (2) Bimzelx® UCB Pharma GmbH Skin diseases Psoriatic arthritis, monotherapy or in combination with methotrexate 30,300 100% additional benefit not proven
Bimekizumab (3) Bimzelx® UCB Pharma GmbH Musculoskeletal system diseases Ankylosing spondylitis 16,800 100% additional benefit not proven
Bimekizumab (4) Bimzelx® UCB Pharma GmbH Musculoskeletal system diseases Axial spondyloarthritis, non-radiographic 19,500 100% additional benefit not proven
Selumetinib (2) Koselugo® Alexion Pharma Deutschland GmbH Oncological diseases Neurofibromatosis (≥ 3 to < 18 years, type 1) 0
510–740 		100% Hint for non-quantifiable additional benefit Orphan repealed
Sacubitril / Valsartan (2) Entresto® Novartis Pharma GmbH Cardiovascular diseases Chronic heart failure with left ventricular dysfunction, ≥ 1 year) 170–860 100% additional benefit not proven
Secukinumab (9) Cosentyx® Novartis Pharma GmbH Skin diseases Hidradenitis suppurativa (Acne inversa) 4,800–6,400 100% additional benefit not proven
Ravulizumab (5) Ultomiris® Alexion Pharma Germany GmbH Nervous system diseases Neuromyelitis optica spectrum disorders, anti-aquaporin-4 IgG seropositive 460–1,170 100% additional benefit not proven
Lisocabtagen maraleucel (2) Breyanzi® Bristol-Myers Squibb GmbH Oncological diseases Diffuse large B-cell lymphoma, highly malignant B-cell lymphoma, primary mediastinal large B-cell lymphoma and follicular lymphoma grade 3B; after 1 prior therapy, relapse within 12 months or refractory) 1,675–2,355 50% Hint for considerable additional benefit
Setmelanotid (2) Imcivree® Rhythm Pharmaceuticals Inc. Metabolic diseases Obesity and control of hunger, Bardet-Biedl syndrome, ≥ 6 years 300–1,100 100% Hint for non-quantifiable additional benefit Orphan
Loncastuximab tesirin (1) Zynlonta® Swedish Orphan Biovitrum GmbH Oncological diseases Diffuse large B-cell lymphoma (DLBCL) and highly malignant B-cell lymphoma (HGBL), ≥ 2 prior therapies) 1,360–1,900 100% additional benefit not proven
Luspatercept (4) Reblozyl® Bristol-Myers Squibb GmbH & Co. KGaA Hematopoietic diseases Β-thalassaemia, transfusion-dependent anaemia 250–330 100% additional benefit not proven Orphan (turnover limit)
Luspatercept (5) Reblozyl® Bristol-Myers Squibb GmbH & Co. KGaA Hematopoietic diseases Myelodysplastic syndrome with transfusion-dependent anaemia, pre-treated 790–1,860 100% additional benefit not proven Orphan (turnover limit)
Tixagevimab / Cilgavimab (2) Evusheld® AstraZeneca GmbH Infectious diseases COVID-19, Preexposition prophylaxis, ≥ 12 years n.d. 100% additional benefit not proven
Cemiplimab (5) Libtayo® Sanofi-Aventis Deutschland GmbH Oncological diseases Non-small cell lung cancer, first-line, PD-L1 expression ≥ 1 %, combination with platinum-based chemotherapy 9,540–12,900 100% additional benefit not proven
Cemiplimab (4) Libtayo® Sanofi-Aventis Deutschland GmbH Oncological diseases Cervix carcinoma, pretreated 380–1,450 50% Indication of considerable additional benefit
Etranacogen Dezaparvovec (1) Hemgenix® CSL Behring GmbH Hematopoietic diseases Hemophilia B 231–358 100% Hint for non-quantifiable additional benefit Orphan
Upadacitinib (7) Rinvoq® AbbVie Deutschland GmbH & Co. KG Digestive system diseases Morbus Crohn, pretreated 18,900–35,350 100% additional benefit not proven
Lasmiditan (1) Rayvow® Lilly Deutschland GmbH Nervous system diseases Migraine acute therapy 2,750,000 100% additional benefit not proven
Tabelecleucel (1) Ebvallo® Pierre Fabre Pharma GmbH Oncological diseases Relapsed or refractory Epstein-Barr virus positive posttransplant lymphoproliferative disease (EBV+ PTLD), pretreated, 2 years and older 7–30 100% Hint for non-quantifiable additional benefit Orphan
Deucravacitinib (1) Sotyktu® Bristol-Myers Squibb GmbH & Co. KGaA Skin diseases Moderate to severe plaque psoriasis 26,900–27,900 100% additional benefit not proven
Belantamab-Mafodotin (2) Blenrep® GlaxoSmithKline GmbH & Co. KG Oncological diseases Multiple myeloma, at least 4 prior therapies, monotherapy 0
570–1,130 		100% Hint for non-quantifiable additional benefit Orphan repealed
Durvalumab (3) Imfinzi® AstraZeneca GmbH Oncological diseases Biliary tumors, first-line, combination with gemcitabine and cisplatin 1,800 100% Indication of minor additional benefit
Durvalumab (4) Imfinzi® AstraZeneca GmbH Oncological diseases Non-small cell lung cancer, EGFR/ALK-negative, first-line, combination with tremelimumab and platinum-based chemotherapy 14,470–24,660 100% additional benefit not proven
Durvalumab (5) Imfinzi® AstraZeneca GmbH Oncological diseases Hepatocellular carcinoma, first-line, combination with tremelimumab 1,900–5,470 100% additional benefit not proven
Tremelimumab (Tremelimumab AstraZeneca, 1) Tremelimumab AstraZeneca® AstraZeneca GmbH Oncological diseases Non-small cell lung cancer, EGFR/ALK-negative, first-line, combination with durvalumab and platinum-based chemotherapy 14,470–24,660 100% additional benefit not proven
Tremelimumab (1) Imjudo® AstraZeneca GmbH Oncological diseases Hepatocellular carcinoma, first-line, combination with durvalumab 1,900–5,470 100% additional benefit not proven
Dupilumab (7) Dupixent® Sanofi-Aventis Deutschland GmbH Skin diseases Prurigo nodularis 3,500–4,800 100% Hint for non-quantifiable additional benefit
Darolutamid (2) Nubeqa® Bayer Vital GmbH Oncological diseases Prostate carcinoma, metastatic, hormone-sensitive, combination with docetaxel and androgen deprivation therapy 2,590–3,640 100% Indication of considerable additional benefit
Olaparib (11) Lynparza® AstraZeneca GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, BRCA-mutated, FIGO stages III and IV, maintenance therapy 520–630 100% additional benefit not proven
Pitolisant (2) Wakix® Bioprojet Deutschland GmbH Nervous system diseases Narcolepsy, with or without cataplexy (children and adolescents, 6-17 years) 120–650 100% Hint for non-quantifiable additional benefit Orphan
Dulaglutid (3) Trulicity® Lilly Deutschland GmbH Metabolic diseases Diabetes mellitus type 2, ≥ 10 years 640–710 100% additional benefit not proven
Luspatercept (3) Reblozyl® Bristol-Myers Squibb GmbH & Co. KGaA Hematopoietic diseases Β-thalassaemia, non-transfusion-dependent anaemia 470–560 100% Indication of minor additional benefit Orphan (turnover limit)
Rucaparib (3) Rubraca® Zr pharma& GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, maintenance therapy 1,750–2,270 100% additional benefit not proven
Dupilumab (8) Dupixent® Sanofi-Aventis Deutschland GmbH Digestive system diseases Eosinophilic oesophagitis, ≥ 12 years, min. 40 kg 3,900–4,400 100% additional benefit not proven
Dupilumab (9) Dupixent® Sanofi-Aventis Deutschland GmbH Skin diseases Atopic dermatitis, 6 months to 5 years 2,700–3,900 50% Hint for non-quantifiable additional benefit
Esketamin (3) Spravato® Janssen-Cilag GmbH Mental illnesses Depression, therapy resistent, in combination with SSRI or SNRI 317,000–505,000 100% Hint for considerable additional benefit
Voclosporin (1) Lupkynis® Otsuka Pharma GmbH Musculoskeletal system diseases Lupusnephritis 1,090–13,050 100% additional benefit not proven
Emicizumab (3) Hemlibra® Roche Pharma AG Hematopoietic diseases Moderate haemophilia A, without factor VIII inhibitors, with severe bleeding phenotype 220–240 100% additional benefit not proven
Finerenon (2) Kerendia® Bayer Vital GmbH Genitourinary system diseases Chronic kidney disease in type 2 diabetes, stages 1 and 2 with albuminuria 436,400–493,750 100% Hint for non-quantifiable additional benefit
Finerenon (1) Kerendia® Bayer Vital GmbH Genitourinary system diseases Chronic kidney disease in type 2 diabetes, stages 3 and 4 with albuminuria 304,500–322,500 100% additional benefit not proven
Dolutegravir / Abacavir / Lamivudin (2) Triumeq® ViiV Healthcare GmbH Infectious diseases HIV infection, ≥ 14 kg to < 12 years 86 100% additional benefit not proven
Dapagliflozin (8) Forxiga® AstraZeneca GmbH Cardiovascular diseases Chronic heart failure with left ventricular ejection fraction LVEF > 40 % 1,270,000–1,400,000 100% Hint for minor additional benefit
Ciltacabtagen autoleucel (1) Carvykti® Janssen-Cilag GmbH Oncological diseases Multiple myeloma, at least 3 previous therapies 0
1,210–1,310 		100% Hint for non-quantifiable additional benefit Orphan repealed
Fenfluramin (2) Fintepla® Zogenix GmbH Nervous system diseases Lennox-Gastaut syndrome, add-on therapy, ≥ 2 years 2,100–22,700 100% Hint for considerable additional benefit Orphan
Sotorasib (2) Lumykras® Amgen Europe B.V. Oncological diseases Non-small cell lung cancer (NSCLC), KRAS G12C mutation, ≥ 1 prior therapy 480–1,040 44% Hint for non-quantifiable additional benefit
Trastuzumab deruxtecan (3) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases Adenocarcinoma (AC) of the stomach or gastro-oesophageal junction, HER2-positive, after trastuzumab-based therapy 110–170
470–770 		100% additional benefit not proven repealed subpopulations
Trastuzumab deruxtecan (4) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases Breast carcinoma (BC), HER2-low, pre-treated 1,350–4,700 100% Indication of considerable additional benefit
Ibrutinib (9) Imbruvica® Janssen-Cilag GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), first-line, combination with venetoclax 3,190–3,200 100% additional benefit not proven
Spesolimab (1) Spevigo® Boehringer Ingelheim Pharma GmbH & Co. KG Skin diseases Generalised pustular psoriasis, acute treatment 170–400 100% additional benefit not proven
Lutetium (177Lu) Vipivotidtetraxetan (1) Pluvicto® Novartis Radiopharmaceuticals GmbH Oncological diseases Prostate carcinoma (PC), combination with androgen deprivation therapy, PSMA-positive, metastatic, castration-resistant, progression after inhibition of the AR pathway and taxane-based chemotherapy 1,500–2,400 50% Indication of considerable additional benefit
Maralixibat (1) Livmarli® Mirum Pharmaceuticals Germany GmbH Digestive system diseases Alagille-Syndrome, ≥ 2 months 139–377 100% Hint for non-quantifiable additional benefit Orphan
Olaparib (10) Lynparza® AstraZeneca GmbH Oncological diseases Prostate carcinoma (PC), metastatic, castration-resistant, chemotherapy not clinically indicated, combination with abiraterone and/or prednisone 9,400–12,200 25% Hint for considerable additional benefit
Diroximelfumarat (1) Vumerity® Biogen GmbH Nervous system diseases Multiple Sclerosis (MS) n.d. discontinued
Risankizumab (3) Skyrizi® AbbVie Deutschland GmbH & Co. KG Digestive system diseases Morbus Crohn, pre-treated 18,800–35,250 40% Hint for minor additional benefit
Bictegravir / Emtricitabin / Tenofoviralafenamid (2) Biktarvy® Gilead Sciences GmbH Infectious diseases HIV-Infection, 2 until < 18 years 184 100% additional benefit not proven
Sutimlimab (1) Enjaymo® Sanofi-Aventis Deutschland GmbH Hematopoietic diseases Cold agglutinin disease (CAD) 370–1,510 100% Hint for minor additional benefit Orphan
Abemaciclib (6) Verzenios® Lilly Deutschland GmbH Oncological diseases Breast carcinoma (BC), HR+, HER2-, combination with aromatase inhibitor 7,400–34,790 100% Hint for minor additional benefit
Latanoprost / Netarsudil (1) Roclanda® Santen GmbH Eye diseases Reduction of increased intraocular pressure in open-angle glaucoma or ocular hypertension 87,800 100% additional benefit not proven
Zanubrutinib (2) Brukinsa® BeiGene Germany GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), first-line 3,180–3,200 50% Hint for minor additional benefit
Zanubrutinib (3) Brukinsa® BeiGene Germany GmbH Oncological diseases Marginal zone lymphoma (MZL), after at least 1 previous therapy with anti-CD20 antibody 590–1,760 100% additional benefit not proven
Zanubrutinib (4) Brukinsa® BeiGene Germany GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), relapsed and/or refractory 8,800–12,750 23% Indication of minor additional benefit
Olopatadin / Mometason (1) Ryaltris® Berlin-Chemie AG Respiratory system diseases Allergic rhinitis 3,807,000–7,356,000 100% additional benefit not proven
Maribavir (1) Livtencity® Takeda GmbH Infectious diseases Cytomegalovirus infection (CMI), refractory to therapy 90–130 100% Hint for minor additional benefit Orphan
Tezepelumab (1) Tezspire® AstraZeneca GmbH Respiratory system diseases Bronchial asthma (AB), ≥ 12 years 42,300–46,700 100% additional benefit not proven
Tralokinumab (2) Adtralza® LEO Pharma GmbH Skin diseases Atopic dermatitis (AD), 12 to < 18 years 5,300–10,600 100% additional benefit not proven
Daridorexant (1) Quviviq® Idorsia Pharmaceuticals Germany GmbH Nervous system diseases Sleep disorders (mental illnesses) 0
1,900–79,000 		100% additional benefit not proven repealed
Dalbavancin (1) Xydalba® Advanz Pharma Germany GmbH Infectious diseases Acute bacterial skin and skin structure infections (ABSSSI) n.d. n.d.
Elvitegravir / Cobicistat / Emtricitabin / Tenofoviralafenamid (3) Genvoya® Gilead Sciences GmbH Infectious diseases HIV infection, 2 to < 6 years 16 100% additional benefit not proven
Olaparib (9) Lynparza® AstraZeneca GmbH Oncological diseases Ovarian carcinoma (OC), fallopian tube carcinoma or primary peritoneal carcinoma, FIGO stages III and IV, HRD-positive, maintenance therapy, combination with bevacizumab 1,030–1,250 100% Hint for considerable additional benefit
Ravulizumab (4) Ultomiris® Alexion Pharma Germany GmbH Nervous system diseases Myasthenia gravis, AChR antibody+ 800–1,200 100% additional benefit not proven
Tixagevimab / Cilgavimab (1) Evusheld® AstraZeneca GmbH Infectious diseases COVID-19, erhöhtes Risiko für schweren Verlauf, ≥ 12 Jahre n.d. 100% Hint for minor additional benefit
Faricimab (2) Vabysmo® Roche Pharma AG Eye diseases Neovascular age-related macular degeneration (AMD) 85,200–681,400 100% additional benefit not proven
Remdesivir (4) Veklury® Gilead Sciences GmbH Infectious diseases COVID-19, no requirement for supplementary oxygen, < 18 years, ≥ 40 kg) 2,600–14,200 100% additional benefit not proven
Remdesivir (3) Veklury® Gilead Sciences GmbH Infectious diseases COVID-19, need for supplementary oxygen, ≥ 4 weeks - < 12 years 350–1,060 100% additional benefit not proven
Vutrisiran (1) Amvuttra® Alnylam Germany GmbH Metabolic diseases Hereditary transthyretin amyloidosis (hTTA) with polyneuropathy (stage 1 or 2) 360 100% Indication of minor additional benefit Orphan
Relugolix (1) Orgovyx® Accord Healthcare GmbH Oncological diseases Prostate carcinoma (PC), advanced, hormone-sensitive 25,020–44,280 100% additional benefit not proven
Lonafarnib (1) Zokinvy® EigerBio Europe Ltd. Other diseases Hutchinson-Gilford progeria syndrome or progeroid laminopathy, 12 months and over 5–6 100% Hint for non-quantifiable additional benefit Orphan
Faricimab (1) Vabysmo® Roche Pharma AG Eye diseases Diabetic macular edema (DME) 190,000–241,000 100% additional benefit not proven
Difelikefalin (1) Kapruvia® Fresenius Medical Care Nephrologica Deutschland GmbH Skin diseases Pruritus in chronic kidney disease (CKD), haemodialysis patients 3,500–22,000 100% additional benefit not proven
Lisocabtagen maraleucel (1) Breyanzi® Bristol-Myers Squibb GmbH Oncological diseases Diffuse large B-cell lymphoma (DLBCL), primary mediastinal large B-cell lymphoma and follicular lymphoma (PMBCL) grade 3B, after ≥ 2 prior therapies 1,420–1,980 100% additional benefit not proven
Selinexor (1) Nexpovio® Stemline Therapeutics B.V. Oncological diseases Multiple myeloma (MM) at least 1 prior therapy, combination with bortezomib and dexamethasone 4,700–7,000 100% additional benefit not proven
Selinexor (2) Nexpovio® Stemline Therapeutics B.V. Oncological diseases Multiple myeloma (MM) at least 4 prior therapies, combination with dexamethasone 570–1,130 100% additional benefit not proven
Asciminib (1) Scemblix® Novartis Pharma GmbH Oncological diseases Chronic myeloid leukaemia (CML), Ph+, after ≥ 2 prior therapies 0
840–1,150 		100% Indication of minor additional benefit Orphan repealed
Melphalanflufenamid (1) Pepaxti® Oncopeptides AB Oncological diseases Multiple myeloma (MM) after at least 3 previous therapies, combination with dexamethasone) 1,200–1,300 100% additional benefit not proven
Olipudase alfa (1) Xenpozyme® Sanofi-Aventis Deutschland GmbH Metabolic diseases Acid sphingomyelinase deficiency (ASMD) type A/B or type B 70–80 100% Hint for non-quantifiable additional benefit Orphan
Pertuzumab (4) Perjeta® Roche Pharma AG Oncological diseases Breast carcinoma (BC), early with high risk of recurrence, adjuvant therapy, combination with trastuzumab and chemotherapy 1,910–3,060 100% Indication of minor additional benefit
Pertuzumab / Trastuzumab (4) Phesgo® Roche Pharma AG Oncological diseases Breast carcinoma (BC), HER2+, early with high risk of recurrence, adjuvant therapy, combination with chemotherapy 1,910–3,060 100% Indication of minor additional benefit
Selpercatinib (5) Retsevmo® Lilly Deutschland GmbH Oncological diseases Thyroid carcinoma (MTC), RET-mutated, monotherapy, 12 years and older 0
40–170 		100% additional benefit not proven repealed
Brexucabtagen-Autoleucel / Autologe Anti-CD19-transduzierte CD3+ Zellen (2) Tecartus® Gilead Sciences GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL), aged > 26 81–200 100% Hint for non-quantifiable additional benefit Orphan
Valoctocogen Roxaparvovec (1) Roctavian® BioMarin International Ltd. Hematopoietic diseases Hemophilia A 690–800 100% Hint for non-quantifiable additional benefit Orphan
Burosumab (6) Crysvita® Kyowa Kirin GmbH Metabolic diseases FGF23-related hypophosphatemia in tumour-induced osteomalacia 60–140 100% additional benefit not proven Orphan (turnover limit)
Efgartigimod alfa (1) Vyvgart® Argenx Germany GmbH Nervous system diseases Myasthenia gravis, AChR antibody+ 0
14,000–16,800 		100% Hint for considerable additional benefit Orphan repealed
Eptinezumab (1) Vyepti® Lundbeck GmbH Nervous system diseases Migraine prophylaxis 1,614,300–1,645,200 100% additional benefit not proven
Olaparib (8) Lynparza® AstraZeneca GmbH Oncological diseases Breast carcinoma (BC), HER2, BRCA1/2 mutation, pre-treated, high risk of recurrence, adjuvant, monotherapy or combination with chemotherapy. 540–690 100% Indication of minor additional benefit
Upadacitinib (5) Rinvoq® AbbVie Deutschland GmbH & Co. KG Digestive system diseases Ulcerative colitis (UC), pre-treated 5,300–25,000 100% additional benefit not proven
Upadacitinib (6) Rinvoq® AbbVie Deutschland GmbH & Co. KG Musculoskeletal system diseases Axial spondyloarthritis (AS), non-radiographic 19,500 100% additional benefit not proven
Birkenrindenextrakt (1) Filsuvez® Amryt Pharma GmbH Other diseases Wound treatment in epidermolysis bullosa (from 6 months) 270–860 100% Hint for minor additional benefit Orphan
Ceftolozan / Tazobactam (7) Zerbaxa® MSD SHARP & DOHME GMBH Infectious diseases Bacterial infections, multiple indications, < 18 years of age 40 100% additional benefit considered proven
Capmatinib (1) Tabrecta® Novartis Pharma GmbH Oncological diseases Non-small cell lung cancer (NSCLC), METex14 skipping mutation, pre-treated patients 540–900 100% additional benefit not proven
Eladocagene exuparvovec (1) Upstaza® PTC Therapeutics International Limited Metabolic diseases Aromatic L-amino acid decarboxylase (AADC) deficiency, ≥ 18 months 4–30 100% Hint for non-quantifiable additional benefit Orphan
Trastuzumab deruxtecan (1) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases Breast carcinoma (BC), HER2+, after 1 prior therapy 3,370–3,750 100% Indication of non-quantifiable additional benefit
Trastuzumab deruxtecan (2) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases Breast carcinoma (BC), HER2+, at least 2 previous therapies 1,350–1,640 100% Indication of considerable additional benefit
Pembrolizumab (28) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Cervical carcinoma, PD-L1 expression ≥ 1 (CPS), combination with or without bevacizumab 1,315–1,525 40% Indication of considerable additional benefit
Pembrolizumab (22) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Renal cell carcinoma (RCC), adjuvant, monotherapy, pre-treated patients 1,518–1,973 100% Hint for minor additional benefit
Pembrolizumab (23) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Colorectal cancer (CRC)) with MSI-H or with dMMR, after fluoropyrimidine-based combination therapy. 350–1,470 100% additional benefit not proven
Pembrolizumab (24) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Endometrial carcinoma with MSI-H or with dMMR, pre-treated 230–3,360 100% additional benefit not proven
Pembrolizumab (25) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Gastric carcinoma with MSI-H or dMMR, pre-treated 80–110 74% Hint for non-quantifiable additional benefit
Pembrolizumab (26) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Small bowel carcinoma with MSI-H or dMMR, pre-treated 40–380 100% additional benefit not proven
Pembrolizumab (27) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Biliary carcinoma with MSI-H or dMMR, pre-treated 20–150 100% additional benefit not proven
Pembrolizumab (29) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Melanoma, adjuvant therapy, ≥ 12 years, monotherapy 1,621–2,314 100% Indication of non-quantifiable additional benefit
Pembrolizumab (30) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Melanoma, ≥ 12 to < 18 years 1–4 100% additional benefit not proven
Glycopyrronium (1) Axhidrox® Dr. August Wolff GmbH & Co. KG Arzneimittel Other diseases Severe primary axillary hyperhidrosis 73,000–704,000 100% additional benefit not proven
Inebilizumab (1) Uplizna® Horizon Therapeutics GmbH Nervous system diseases Neuromyelitis optica spectrum disorders, anti-aquaporin-4 IgG seropositive 460–980 100% additional benefit not proven
Eravacyclin (1) Xerava® PAION Deutschland GmbH Infectious diseases Reserve antibiotics: Complicated intra-abdominal infections (cIAI) 2,600–6,600 100% additional benefit considered proven
Secukinumab (8) Cosentyx® Novartis Pharma GmbH Skin diseases Juvenile psoriatic arthritis (PA), ≥ 6 years 120–180 100% additional benefit not proven
Secukinumab (7) Cosentyx® Novartis Pharma GmbH Musculoskeletal system diseases Enthesitis-related arthritis (ERA) 240–290 100% additional benefit not proven
Atezolizumab (10) Tecentriq® Roche Pharma AG Oncological diseases Non-small cell lung cancer (NSCLC), PD-L1 expression ≥ 50 % of TC, EGFR/ALK negative, adjuvant therapy after resection and chemotherapy 0
700–790 		100% Hint for non-quantifiable additional benefit repealed
Mosunetuzumab (1) Lunsumio® Roche Pharma AG Oncological diseases Follicular lymphoma (FL), after ≥ 2 prior therapies 650–690 100% Hint for non-quantifiable additional benefit Orphan
Pembrolizumab (21) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Breast carcinoma (BC), triple-negative (TNBC), high risk of recurrence, neoadjuvant and adjuvant therapy, monotherapy or combination with chemotherapy 0
2,440–2,520 		50% Hint for minor additional benefit repealed
Selpercatinib (4) Retsevmo® Lilly Deutschland GmbH Oncological diseases Non-small cell lung cancer (NSCLC), RET fusion+, first-line 0
115–310 		100% additional benefit not proven repealed
Palbociclib (3) Ibrance® Pfizer Pharma GmbH Oncological diseases Breast carcinoma (BC), patient population a1 7,400–34,700 100% additional benefit not proven
Nirmatrelvir / Ritonavir (1) Paxlovid® Pfizer Pharma GmbH Infectious diseases COVID-19, no need for supplementary oxygen, increased risk of severe progression 218,000–1,307,000 100% Hint for considerable additional benefit
Cerliponase alfa (2) Brineura® BioMarin Deutschland GmbH Metabolic diseases Neuronal ceroid lipofuscinosis (NCL) type 2 40–50 100% Hint for major additional benefit Orphan
Enfortumab Vedotin (1) Padcev® Astellas Pharma Europe B.V. Oncological diseases Urothelial carcinoma (UC) pre-treated with platinum-based chemotherapy and PD-(L)1 inhibitor 410–1,250 47% Hint for considerable additional benefit
Setmelanotid (1) Imcivree® Rhythm Pharmaceuticals Inc. Metabolic diseases Obesity and control of hunger, POMC-, PCSK1- or LEPR-deficient obesity, ≥ 6 years 140–280 100% Hint for non-quantifiable additional benefit Orphan
Cabozantinib (Cabometyx, 6) Cabometyx® Ipsen Pharma GmbH Oncological diseases Thyroid carcinoma (MTC), refractory to radioiodine, pre-treated patients 125–425 100% additional benefit not proven
Polatuzumab Vedotin (2) Polivy® Roche Pharma AG Oncological diseases Diffuse large B-cell lymphoma (DLBCL), combination with rituximab, cyclophosphamide, doxorubicin and prednisone (R-CHP) 0
5,510–6,130 		100% Hint for non-quantifiable additional benefit Orphan repealed
Tisagenlecleucel (5) Kymriah® Novartis Pharma GmbH Oncological diseases Follicular lymphoma (FL), pre-treated patients 650–690 100% Hint for non-quantifiable additional benefit Orphan
Axicabtagen-Ciloleucel (3) Yescarta® Gilead Sciences GmbH Oncological diseases Diffuse large B-cell lymphoma (DLBCL) and primary mediastinal large B-cell lymphoma, after at least 2 prior therapies 0
455–729 		100% Hint for non-quantifiable additional benefit Orphan repealed
Voxelotor (1) Oxbryta® Global Blood Therapeutics Germany GmbH Hematopoietic diseases Haemolytic anaemia in sickle cell disease, monotherapy or combination with hydroxycarbamide, ≥ 12 years 1,590–2,580 100% Hint for non-quantifiable additional benefit Orphan
Sotrovimab (1) Xevudy® GlaxoSmithKline GmbH & Co. KG Infectious diseases COVID-19, ≥ 12 years n.d. 100% Hint for considerable additional benefit
Imipenem / Cilastatin / Relebactam (2) Recarbrio® MSD Sharp & Dohme GmbH Infectious diseases Bacterial infections, multiple indications 2,598–6,600 100% additional benefit considered proven
Ceftolozan / Tazobactam (6) Zerbaxa® MSD Sharp & Dohme GmbH Infectious diseases Bacterial infections, multiple indications 2,600–6,600 100% additional benefit considered proven
Ceftazidim / Avibactam (1) Zavicefta® Pfizer Pharma GmbH Infectious diseases Bacterial infections, multiple indications 2,600–6,600 100% additional benefit considered proven
Tebentafusp (1) Kimmtrak® Immunocore Ireland Ltd. Oncological diseases Uveal melanoma, HLA-A*02:01-positive 0
110 		100% Hint for considerable additional benefit Orphan repealed
Brolucizumab (2) Beovu® Novartis Pharma GmbH Eye diseases Diabetic macular edema 190,000–241,000 100% additional benefit not proven
Abemaciclib (5) Verzenios® Lilly Deutschland GmbH Oncological diseases Breast cancer (BC), HR+, HER2-, early with high risk of recurrence, adjuvant, combination with endocrine therapy 6,905–9,435 40% Hint for minor additional benefit
Doravirin / Lamivudin / Tenofovirdisoproxil (2) Delstrigo® MSD Sharp & Dohme GmbH Infectious diseases HIV infection, 12 to < 18 years 10–20 100% additional benefit not proven
Doravirin (2) Pifeltro® MSD Sharp & Dohme GmbH Infectious diseases HIV infection, 12 to < 18 years 130–140 100% additional benefit not proven
Nivolumab (21) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Urothelial carcinoma (UC) PD-L1 expression ≥ 1 %, adjuvant therapy 350–460
1,030–1,290 		65% Hint for non-quantifiable additional benefit repealed subpopulations
Nivolumab (22) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Squamous cell carcinoma of the oesophagus, PD-L1 expression ≥ 1, first-line, combination with platinum- and fluoropyrimidine-based chemotherapy 920–1,580 100% Indication of considerable additional benefit
Nivolumab (23) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Squamous cell carcinoma (SCC) of the oesophagus, PD-L1 expression ≥ 1, first-line, combination with ipilimumab 920–1,560 100% Hint for considerable additional benefit
Casirivimab / Imdevimab (1) Ronapreve® Roche Pharma AG Infectious diseases Post-exposure prophylaxis of COVID-19 infection, ≥ 12 years n.d. 100% Hint for minor additional benefit repealed
Casirivimab / Imdevimab (2) Ronapreve® Roche Pharma AG Infectious diseases COVID-19-Infection, ≥ 12 years n.d. 100% Hint for considerable additional benefit repealed
Dupilumab (6) Dupixent® Sanofi-Aventis Deutschland GmbH Respiratory system diseases Asthma bronchiale, ≥ 6 until ≤ 11 years 150–860 100% additional benefit not proven
Glucarpidase (1) Voraxaze® SERB GmbH Other diseases Reduction of toxic MTX plasma concentrations; children ≥ 28 days and adults. 90–440 100% Hint for non-quantifiable additional benefit Orphan
Anifrolumab (1) Saphnelo® AstraZeneca GmbH Musculoskeletal system diseases Systemic lupus erythematosus (SLE) 4,600–18,500 100% additional benefit not proven
Avapritinib (2) Ayvakyt® Blueprint Medicines (Germany) GmbH Oncological diseases Systemic mastocytosis, after at least 1 prior therapy 0
270–680 		100% Hint for non-quantifiable additional benefit Orphan repealed
Somatrogon (1) Ngenla® Pfizer Pharma GmbH Metabolic diseases Growth disturbance due to growth hormone deficiency, ≥ 3 to < 18 years of age 5,710–6,550 100% Hint for non-quantifiable additional benefit Orphan
Dolutegravir (4) Tivicay® ViiV Healthcare GmbH Infectious diseases HIV infection, age 6 to < 18 years n.d. n.d.
Voretigen Neparvovec (2) Luxturna® Novartis Pharma GmbH Eye diseases Hereditary retinal dystrophy 100–530 100% Hint for considerable additional benefit Orphan
Pegcetacoplan (1) Aspaveli® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Paroxysmal nocturnal haemoglobinuria (PNH), pre-treated patients 190–520 100% Hint for non-quantifiable additional benefit Orphan
Idebenon (2) Raxone® Chiesi GmbH Eye diseases Leber's hereditary optic neuropathy (LHOP) 1,400–3,000 100% Hint for non-quantifiable additional benefit Orphan
Empagliflozin (4) Jardiance® Boehringer Ingelheim Pharma GmbH & Co. KG Cardiovascular diseases Chronic heart failure (CHF) with preserved ejection fraction 1,270,000–1,400,000 100% Hint for minor additional benefit
Daratumumab (10) Darzalex® Janssen Oncological diseases Multiple myeloma (MM), after at least 1 previous therapy, combination with lenalidomide and dexamethasone or bortezomib and dexamethasone 4,700–7,000 100% Proof of considerable additional benefit Orphan (turnover limit)
Brivaracetam (3) Briviact® UCB Pharma GmbH Nervous system diseases Focal seizures in epilepsy, additional therapy, 2 to < 4 years 300–1,800 100% additional benefit not proven
Lorlatinib (2) Lorviqua® Pfizer Pharma GmbH Oncological diseases Non-small cell lung cancer (NSCLC), ALK+, first-line 390–1,310 100% additional benefit not proven
Tepotinib (1) Tepmetko® Merck Europe B.V. Oncological diseases Non-small cell lung cancer (NSCLC), METex14 skipping, pre-treated patients 540–910 100% additional benefit not proven
Vedolizumab (2) Entyvio® Takeda GmbH Digestive system diseases Antibiotic-refractory pouchitis, pre-treated patients 20–40 100% additional benefit not proven
Ivacaftor / Tezacaftor / Elexacaftor (10) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), F508del mutation, heterozygous and other or unknown mutation, ≥ 6 to ≤ 11 years, combination with ivacaftor 56 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (9) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), F508del mutation, heterozygous and RF mutation, ≥ 6 to ≤ 11 years, combination with ivacaftor 21 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (8) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis, F508del mutation, heterozygous and gating mutation, ≥ 6 to ≤ 11 years, combination with ivacaftor) 28 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (7) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor, 6 to ≤ 11 years (homozygous for F508del mutation) 470 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (6) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor, from 6 to ≤ 11 years (heterozygous for F508del and MF mutation) 233 100% Indication of considerable additional benefit Orphan (turnover limit)
Ivacaftor (27) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), F508del mutation, heterozygous and other or unknown mutation, ≥ 6 to ≤ 11 years, combination with ivacaftor / tezacaftor / elexacaftor 56 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (26) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), F508del mutation, heterozygous and RF mutation, ≥ 6 to ≤ 11 years, combination with ivacaftor / tezacaftor / elexacaftor 21 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (25) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), F508del mutation, heterozygous and gating mutation, ≥ 6 to ≤ 11 years, combination with ivacaftor / tezacaftor / elexacaftor). 28 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (24) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor/tezacaftor/elexacaftor, 6 to ≤ 11 years (homozygous for F508del mutation) 470 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor (23) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor/tezacaftor/elexacaftor, 6 to ≤ 11 years (heterozygous for F508del and MF mutation) 230 100% Indication of considerable additional benefit Orphan (turnover limit)
Avacopan (1) Tavneos® Vifor Pharma Deutschland GmbH Musculoskeletal system diseases Granulomatosis with polyangiitis or microscopic polyangiitis, combination with rituximab or cyclophosphamide 0
2,180–2,280 		100% Hint for minor additional benefit Orphan repealed
Sotorasib (1) Lumykras® Amgen Europe B.V. Oncological diseases Non-small cell lung cancer (NSCLC), KRAS G12C mutation, ≥ 1 therapies 500–1,080
560–1,210 		100% additional benefit not proven repealed subpopulations
Sofosbuvir / Velpatasvir (3) Epclusa® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C, ≥ 3 to < 6 years of age 1–12 100% additional benefit not proven
Calcifediol (1) Rayaldee® Fresenius Medical Care Nephrologica Deutschland GmbH Metabolic diseases Secondary hyperparathyroidism in chronic renal failure 146,700–184,300 100% additional benefit not proven
Isofluran (1) Sedaconda® Sedana Medical AB Other diseases Sedation of mechanically ventilated patients during intensive care therapy (ICT) 146,000–219,000 100% additional benefit not proven
Burosumab (5) Crysvita® Kyowa Kirin GmbH Metabolic diseases X-linked hypophosphatemia, ≥ 18 years of age 410–810 100% additional benefit not proven Orphan (turnover limit)
Burosumab (4) Crysvita® Kyowa Kirin GmbH Metabolic diseases X-linked hypophosphatemia, ≥ 1 to < 18 years of age 200–550 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Duvelisib (2) Copiktra® Secura Bio Limited Oncological diseases Follicular lymphoma (FL), after ≥ 2 prior therapies 380–5,170 100% additional benefit not proven
Duvelisib (1) Copiktra® Secura Bio Limited Oncological diseases Chronic lymphocytic leukemia (CLL), after ≥ 2 prior therapies 550–2,060 100% additional benefit not proven
Abrocitinib (1) Cibinqo® Pfizer Pharma GmbH Skin diseases Atopic Dermatitis (AD) 52,000 100% Hint for considerable additional benefit
Amivantamab (1) Rybrevant® Janssen-Cilag GmbH Oncological diseases Lung cancer, non-small cell (NSCLC), EGFR exon 20 insertion mutation, after platinum-based therapy 9–26 100% additional benefit not proven
Remdesivir (2) Veklury® Gilead Sciences GmbH Infectious diseases COVID-19, no need for supplementary oxygen, increased risk of severe progression 218,000–1,307,000 100% Hint for minor additional benefit
Lenvatinib (Kisplyx, 3) Kisplyx® Eisai GmbH Oncological diseases Advanced renal cell carcinoma (RCC), first-line, combination with pembrolizumab 2,790–4,180 100% additional benefit not proven
Lenvatinib (4) Lenvima® Eisai GmbH Oncological diseases Endometrial carcinoma (EC), after platinum-based therapy, combination with pembrolizumab 1,130–5,070 100% Indication of considerable additional benefit
Pembrolizumab (19) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Endometrial carcinoma (EC) , after platinum-based therapy, combination with lenvatinib 1,130–5,070 100% Indication of considerable additional benefit
Pembrolizumab (20) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Advanced renal cell carcinoma (RCC), first-line, combination with lenvatinib 2,790–4,189 100% additional benefit not proven
Zanubrutinib (1) Brukinsa® BeiGene Germany GmbH Oncological diseases Waldenström's disease, first-line (chemo-immunotherapy unsuitable) or after at least 1 previous therapy 450–1,050 100% additional benefit not proven
Idecabtagen vicleucel (1) Abecma® Bristol-Myers Squibb GmbH & Co KGaA Oncological diseases Multiple myeloma (MM), at least 3 previous therapies 0
1,200–1,300 		100% Hint for non-quantifiable additional benefit Orphan repealed
Evolocumab (3) Repatha® Amgen GmbH Metabolic diseases Primary hypercholesterolaemia or mixed dyslipidaemia, ≥ 10 to < 18 years of age 768–950 100% additional benefit not proven
Ripretinib (1) Qinlock® Deciphera Pharmaceuticals (Netherlands) B.V. Oncological diseases Gastrointestinal stromal tumours (GIST), ≥ 3 prior therapies 220–300 100% Hint for major additional benefit Orphan
Pralsetinib (1) Gavreto® Roche Pharma AG Oncological diseases Non-small cell lung cancer (NSCLC), RET fusion+ 170–510 100% additional benefit not proven
Tofacitinib (7) Xeljanz® Pfizer Pharma GmbH Musculoskeletal system diseases Ankylosing spondylitis (AS) 16,800 100% additional benefit not proven
Ozanimod (2) Zeposia® Bristol-Myers Squibb GmbH & Co. KGaA Digestive system diseases Ulcerative colitis (UC), pre-treated patients 5,300–25,000 100% additional benefit not proven
Dapagliflozin (7) Forxiga® AstraZeneca GmbH Metabolic diseases Diabetes mellitus Type 2, ≥ 10 years 650–710 100% additional benefit not proven
Macimorelinacetate (1) Gryvelin® Consilient Health Ltd. Other diseases Hypohysis function tests n.d. exempted
Ponesimod (2) Ponvory® Janssen-Cilag GmbH Nervous system diseases Relaps multiple sclerosis (MS) 186,000–200,000 50% Indication of minor additional benefit
Abemaciclib (4) Verzenios® Lilly Deutschland GmbH Oncological diseases Breast cancer (BC), HR+, HER2-, combination with fulvestrant 12,870–59,690 42% Indication of minor additional benefit
Filgotinib (2) Jyseleca® Galapagos Biopharma Germany GmbH Digestive system diseases Ulcerative colitis (UC), pretreated patients 5,300–25,000 100% additional benefit not proven
Mepolizumab (4) Nucala® GlaxoSmithKline GmbH & Co. KG Musculoskeletal system diseases Eosinophilic granulomatosis with polyangiitis 80–1,130 100% additional benefit not proven
Mepolizumab (3) Nucala® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Chronic rhinosinusitis with nasal polyps 10,500–12,600 100% additional benefit not proven
Mepolizumab (5) Nucala® GlaxoSmithKline GmbH & Co. KG Hematopoietic diseases Hypereosinophilic syndrome 100–400 100% Hint for considerable additional benefit
Ertugliflozin (1) Steglatro® MSD Sharp & Dohme GmbH Metabolic diseases Diabetes mellitus (DM) type 2 1,451,000–2,021,000 100% additional benefit not proven
Nivolumab (20) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Adenocarcinoma (AC) of the stomach, gastroesophageal junction or esophagus, CPS ≥ 5, HER2-negative, first-line, combination with fluoropyrimidine- and platinum-based combination chemotherapy 500–3,100 100% Hint for considerable additional benefit
Risankizumab (2) Skyrizi® AbbVie Deutschland GmbH & Co. KG Skin diseases Psoriatic arthritis (PA), monotherapy or combination with methotrexate 29,100 100% additional benefit not proven
Sacituzumab govitecan (1) Trodelvy® Gilead Sciences GmbH Oncological diseases Breast cancer (BC) triple-negative, after 2 previous therapies 1,150–2,370 100% Indication of major additional benefit
Lusutrombopag (1) Mulpleo® Shionogi GmbH Hematopoietic diseases Thrombocytopenia in chronic liver disease 1,790–24,130 100% additional benefit not proven
Cefiderocol (2) Fetcroja® Shionogi GmbH Infectious diseases Infections caused by aerobic gram-negative pathogens 2,600–6,600 100% additional benefit considered proven
Delamanid (1) Deltyba® Otsuka Novel Products GmbH Infectious diseases Multidrug-resistant pulmonary tuberculosis (TBC), ≥ 10 kg 80–114 100% Hint for non-quantifiable additional benefit Orphan
Elbasvir / Grazoprevir (2) Zepatier® Merck Sharp & Dohme B.V. Infectious diseases Chronic hepatitis C (HCV), 12 to < 18 years 10–17 100% additional benefit not proven
Pembrolizumab (17) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Carcinoma of the esophagus or gastroesophageal junction, PD-L1 expression ≥ 10 (CPS), first-line, combination with platinum- and fluoropyrimidine-based chemotherapy. 535–805 34% Indication of considerable additional benefit
Pembrolizumab (18) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Breast cancer (BC), triple-negative, PD-L1 expression ≥ 10 (CPS), combination with chemotherapy 260–270 50% Hint for considerable additional benefit
Icosapent-Ethyl (1) Vazkepa® Amarin Pharmaceuticals Ireland Limited Metabolic diseases Dyslipidemia, pre-treated patients 844,000–878,000 100% additional benefit not proven
Pitolisant (Ozawade, 1) Ozawade® Bioprojet Deutschland GmbH Nervous system diseases Daytime sleepiness in obstructive sleep apnea, after previous therapy 200,000–400,000 100% additional benefit not proven
Sofosbuvir / Velpatasvir / Voxilaprevir (2) Vosevi® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C (HCV), 12 to < 18 years 24–39 100% additional benefit not proven
Albutrepenonacog alfa (2) Idelvion® CSL Behring GmbH Hematopoietic diseases Hemophilia B, congenital factor IX deficiency 560–720 100% additional benefit not proven Orphan (turnover limit)
Erdnussprotein als entfettetes Pulver von Arachis hypogaea L., semen (Erdnüsse) (1) Palforzia® Aimmune Therapeutics Germany GmbH Other diseases Peanut allergy, ≥ 4 years 43,900–97,200 100% additional benefit not proven
Solriamfetol (2) Sunosi® Jazz Pharmaceuticals Ireland Ltd. Nervous system diseases Daytime sleepiness in obstructive sleep apnoea, after previous therapy 200,000–400,000 100% additional benefit not proven
Vandetanib (4) Caprelsa® Sanofi-Aventis Deutschland GmbH Oncological diseases Thyroid carcinoma (MTC) 50–670 100% additional benefit not proven
Vosoritid (1) Voxzogo® BioMarin International Limited Other diseases Achondroplasia, ≥ 2 years 0
340–480 		100% Hint for non-quantifiable additional benefit Orphan repealed
Daratumumab (9) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma (MM), newly diagnosed, patients not suitable for autologous stem cell transplantation, combination with lenalidomide and dexamethasone 3,470–3,670 100% Hint for considerable additional benefit Orphan (turnover limit)
Lumacaftor / Ivacaftor (4) Orkambi® Vertex Pharmaceuticals (Europe) Limited Metabolic diseases Cystic fibrosis (CF), homozygous F508del mutation in the CFTR gene, ≥ 2 to ≤ 5 years 290 100% Hint for non-quantifiable additional benefit
Ravulizumab (3) Ultomiris® Alexion Pharma Germany GmbH Hematopoietic diseases Paroxysmal hemoglobinuria, pediatric patients 0–49 100% additional benefit not proven
Tofacitinib (6) Xeljanz® Pfizer Pharma GmbH Musculoskeletal system diseases Polyarticular juvenile idiopathic arthritis, RF+ or RF polyarthritis and dilated oligoarthritis, and juvenile psoriatic arthritis, ≥ 2 years 1,450 100% additional benefit not proven
Roxadustat (1) Evrenzo® Astellas Pharma GmbH Hematopoietic diseases Symptomatic anemia in chronic kidney disease (CKD) 151,000–195,000 100% additional benefit not proven
Bimekizumab (1) Bimzelx® UCB Pharma GmbH Skin diseases Plaque psoriasis (PP) 35,900–121,500 100% Indication of minor additional benefit
Vericiguat (1) Verquvo® Bayer Vital GmbH Cardiovascular diseases Chronic heart failure (CHF) 74,600–530,000 100% Hint for minor additional benefit
Odevixibat (1) Bylvay® Albireo Pharma AB Digestive system diseases Cholestasis, ≥ 6 months 40–110 100% Hint for minor additional benefit Orphan
Tafasitamab (1) Minjuvi® Incyte Biosciences Germany GmbH Oncological diseases Diffuse large B-cell lymphoma (DLBCL), combination with lenalidomide 730–1,560 100% Hint for non-quantifiable additional benefit Orphan
Misoprostol (1) Angusta® Norgine GmbH Other diseases Labor induction 76,500–90,000 100% additional benefit not proven
Nivolumab (19) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Carcinoma of the esophagus and gastro-esophageal junction, pre-treated patients, adjuvant therapy 0
590–860 		100% Indication of non-quantifiable additional benefit repealed
Migalastat (2) Galafold® Amicus Therapeutics GmbH Metabolic diseases Fabry disease, 12 to < 16 years 0
1–19 		100% Hint for non-quantifiable additional benefit Orphan repealed
Tofacitinib (5) Xeljanz® Pfizer Pharma GmbH Musculoskeletal system diseases Rheumatoid arthritis (RA), pretreated patients, monotherapy or combination with methotrexate 27,100–93,500 100% additional benefit not proven
Tirbanibulin (1) Klisyri® Almirall Hermal GmbH Skin diseases Actinic keratosis (AK), Olsen grade I 700,000–1,380,000 100% additional benefit not proven
Relugolix / Estradiol / Norethisteronacetat (1) Ryeqo® Gedeon Richter Pharma GmbH Genitourinary system diseases Uterine myoma 20,160–100,840 50% Hint for considerable additional benefit
Dapagliflozin (6) Forxiga® AstraZeneca GmbH Genitourinary system diseases Chronic kidney disease (CKD) 2,520,200–3,409,200 50% Hint for considerable additional benefit
Upadacitinib (4) Rinvoq® AbbVie Deutschland GmbH & Co. KG Skin diseases Atopic dermatitis (AD), ≥ 12 years 57,300–62,850 33% Indication of considerable additional benefit
Selumetinib (1) Koselugo® AstraZeneca GmbH Oncological diseases Neurofibromatosis (type 1), ≥ 3 to < 18 years 0
510–740 		100% Hint for non-quantifiable additional benefit Orphan repealed
Daratumumab (8) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple Myeloma (MM), at least 1 pretreatment, combination with Pomalidomid and Dexamethason 3,190–3,600 38% Hint for minor additional benefit Orphan (turnover limit)
Imipenem / Cilastatin / Relebactam (1) Recarbrio® MSD Sharp & Dohme GmbH Infectious diseases Pneumonia, Bacteriemia n.d. n.d.
Ceftolozan / Tazobactam (5) Zerbaxa® MSD Sharp & Dohme GmbH Infectious diseases Complicated intra-abdominal infections, acute pyelonephritis, complicated urinary tract infections, hospital-acquired pneumonia (HAP) including ventilator-associated pneumonia (VAP) n.d. n.d.
Teriflunomid (2) Aubagio® Sanofi-Aventis Deutschland GmbH Nervous system diseases Relapsing-remitting multiple sclerosis (MS), 10 to 17 years 350–1,200 100% additional benefit not proven
Blinatumomab (6) Blincyto® Amgen GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL), high-risk first relapse, Ph-, CD19+, 1 to ≤ 18 years 7–30 100% Indication of major additional benefit Orphan
Nivolumab (18) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Colorectal carcinoma (CRC) with mismatch repair deficiency or high microsatellite instability, pre-treated patients, combination with ipilimumab 350–475 100% additional benefit not proven
Cemiplimab (3) Libtayo® Sanofi-Aventis Deutschland GmbH Oncological diseases Basal cell carcinoma (BCC), locally advanced or metastasised 83–155 97% Hint for minor additional benefit
Cemiplimab (2) Libtayo® Sanofi-Aventis Deutschland GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), first-line 4,130–5,110 100% additional benefit not proven
Daratumumab (7) Darzalex® Janssen-Cilag GmbH Oncological diseases Systemic light chain amyloidosis, first-line, combination with cyclophosphamide, bortezomib and dexamethasone 0
440–1,030 		50% Hint for minor additional benefit Orphan (turnover limit) repealed
Satralizumab (1) Enspryng® Roche Pharma AG Nervous system diseases Neuromyelitis optica spectrum disorders (NMOSD), anti-aquaporin-4 IgG seropositive, ≥ 12 years 460–5,050 100% Hint for minor additional benefit Orphan
Empagliflozin (3) Jardiance® Boehringer Ingelheim Pharma GmbH & Co. KG Cardiovascular diseases Chronic heart failure (CHF) 2,061,700–2,273,000 100% Hint for minor additional benefit
Tralokinumab (1) Adtralza® Leo Pharma A/S Skin diseases Atopic dermatitis (AD) 52,000 100% additional benefit not proven
Angiotensin-II-Acetat (1) Giapreza® PAION Deutschland GmbH Cardiovascular diseases Hypotension 3,980–37,625 100% additional benefit not proven
Brentuximab Vedotin (6) Adcetris® Takeda GmbH Oncological diseases Systemic anaplastic large cell lymphoma; sALCL; first-line; combination with cyclophosphamide, doxorubicin and prednisone 125–127 100% Hint for minor additional benefit Orphan
Cabozantinib (2) Cometriq® Ipsen Pharma GmbH Oncological diseases Thyroid carcinoma (MTC) 50–670 100% Hint for non-quantifiable additional benefit Orphan
Glecaprevir / Pibrentasvir (3) Maviret® AbbVie Deutschland GmbH & Co. KG Infectious diseases Chronic hepatitis C, 3 to < 12 years 146–238 100% additional benefit not proven
Nivolumab (17) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Malignant pleural mesothelioma, first-line, combination with ipilimumab 160 50% Indication of considerable additional benefit
Osimertinib (4) Tagrisso® AstraZeneca GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), EGFR mutations, adjuvant therapy 0
1,280–1,860 		50% Indication of non-quantifiable additional benefit repealed
Elotuzumab (3) Empliciti® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Multiple myeloma (MM), at least 2 previous therapies, combination with pomalidomide and dexamethasone 2,500 100% Hint for considerable additional benefit
Dostarlimab (1) Jemperli® GlaxoSmithKline GmbH & Co. KG Oncological diseases Endometrial carcinoma (EC), after platinum-based therapy 230–3,360 100% additional benefit not proven
Tagraxofusp (1) Elzonris® Stemline Therapeutics B.V. Oncological diseases Blastic plasmacytoid dendritic cell neoplasm (BPDCN), first-line 30–90 100% Hint for non-quantifiable additional benefit Orphan
Venetoclax (5) Venclyxto® AbbVie Deutschland GmbH & Co. KG Oncological diseases Acute myeloid leukaemia (AML), combination therapy, first-line 560–840 100% Hint for considerable additional benefit
Berotralstat (1) Orladeyo® BioCryst Pharma Deutschland GmbH Other diseases Hereditary angioedema (HAE), prophylaxis, ≥ 12 years 140–430 100% additional benefit not proven
Ponesimod (1) Ponvory® Janssen-Cilag GmbH Nervous system diseases Relapsing multiple sclerosis (MS) 21,000–23,000 100% additional benefit not proven
Ivacaftor / Tezacaftor / Elexacaftor (5) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥ 12 years (heterozygous for F508del and others or unknown mutation). 310 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (4) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥ 12 years (heterozygous for F508del and RF mutation) 173 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (3) Kaftrio® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients > 12 years (heterozygous for F508del and gating mutation (incl. R117H)). 133 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (22) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor/tezacaftor/elexacaftor in patients ≥ 12 years (heterozygous for F508del and others or unknown mutation) 310 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (21) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor/tezacaftor/elexacaftor in patients ≥ 12 years (heterozygous for F508del and RF mutation) 173 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (20) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor/tezacaftor/elexacaftor in patients ≥ 12 years (heterozygous for F508del and gating mutation (incl. R117H)) 133 100% additional benefit not proven Orphan (turnover limit)
Bosutinib (4) Bosulif® Pfizer Pharma GmbH Oncological diseases Chronic myeloid leukaemia (CML), Ph+, first-line 760–890 100% additional benefit not proven
Enzalutamid (5) Xtandi® Astellas Pharma GmbH Oncological diseases Prostate carcinoma (PC), metastatic, hormone-sensitive, combination with androgen deprivation therapy 2,590–3,640 100% additional benefit not proven
Cenobamat (1) Ontozry® Angelini Pharma Deutschland GmbH Nervous system diseases Epilepsy, focal seizures, after at least 2 previous therapies 52,910–167,470 100% additional benefit not proven
Atezolizumab (9) Tecentriq® Roche Pharma AG Oncological diseases Non-small cell lung carcinoma (NSCLC), PD-L1 expression ≥50 % on TC or ≥10 % on IC, EGFR/ALK negative, first-line 4,520–5,080 100% additional benefit not proven
Isatuximab (1) Sarclisa® Sanofi-Aventis Deutschland GmbH Oncological diseases Multiple myeloma (MM), after at least 2 previous therapies, combination with pomalidomide and dexamethasone 2,500 100% Hint for minor additional benefit
Isatuximab (2) Sarclisa® Sanofi-Aventis Deutschland GmbH Oncological diseases Multiple myeloma (MM), after at least 1 prior therapy, combination with carfilzomib and dexamethasone 4,700–7,000 100% additional benefit not proven
Lanadelumab (2) Takhzyro® Takeda GmbH Other diseases Hereditary angioedema (HAE), prophylaxis, ≥ 12 years 140–430 100% additional benefit not proven Orphan (turnover limit)
Onasemnogen-Abeparvovec (2) Zolgensma® Novartis Gene Therapies EU Limited Nervous system diseases 5q-associated spinal muscular atrophy (SMA) 46–67 100% additional benefit not proven Orphan (turnover limit)
Obinutuzumab (6) Gazyvaro® Roche Pharma AG Oncological diseases Follicular lymphoma (FL), first-line 1,300–1,500 100% additional benefit not proven Orphan (turnover limit)
Obinutuzumab (4) Gazyvaro® Roche Pharma AG Oncological diseases Chronic lymphocytic leukemia (CLL) 820–1,480 100% additional benefit not proven Orphan (turnover limit)
Obinutuzumab (5) Gazyvaro® Roche Pharma AG Oncological diseases Follicular lymphoma (FL) 790–940 100% additional benefit not proven Orphan (turnover limit)
Cannabidiol (5) Epidyolex® GW Pharmaceuticals plc Other diseases Seizures in Tuberous Sclerosis, ≥ 2 years 0
500–2,700 		100% Hint for non-quantifiable additional benefit Orphan repealed
Atidarsagen autotemcel OTL-200 (1) Libmeldy® Orchard Therapeutics GmbH Metabolic diseases Metachromatic leukodystrophy with biallelic mutation in the ARSA gene 2–4 67% Hint for major additional benefit Orphan
Cabotegravir (1) Vocabria® ViiV Healthcare GmbH Infectious diseases HIV-1 infection, pre-treated, combination with rilpivirin 59,900 100% additional benefit not proven
Erenumab (2) Aimovig® Novartis Pharma GmbH Nervous system diseases Migraine prophylaxis 1,540,100–1,568,800 100% Hint for considerable additional benefit
Risdiplam (1) Evrysdi® Roche Pharma AG Nervous system diseases 5q-associated spinal muscular atrophy (SMA) 992–2,142 42% Hint for non-quantifiable additional benefit Orphan
Cabozantinib (Cabometyx, 5) Cabometyx® Ipsen Pharma GmbH Oncological diseases Renal cell carcinoma (RCC), first-line, combination with nivolumab 2,790–4,180 100% additional benefit not proven
Nivolumab (16) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Renal cell carcinoma (RCC), first-line, combination with cabozantinib 2,790–4,180 100% additional benefit not proven
Rilpivirin (Rekambys, 1) Rekambys® ViiV Healthcare GmbH Infectious diseases HIV-1 infection, pre-treated, combination with cabotegravir 59,900 100% additional benefit not proven
Cefiderocol (1) Fetcroja® Shionogi GmbH Infectious diseases Infections caused by aerobic gram-negative pathogens n.d. n.d.
Pemigatinib (1) Pemazyre® Incyte Biosciences Germany GmbH Oncological diseases Cholangiocarcinoma with FGFR2 fusion or FGFR2 rearrangement, at least 1 prior therapy 35–300 100% Hint for non-quantifiable additional benefit Orphan
Nivolumab (15) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, adjuvant therapy 3,450–4,350 100% Hint for considerable additional benefit
Natrium-Zirkonium-Cyclosilikat (1) Lokelma® AstraZeneca GmbH Metabolic diseases Hyperkalemia 77,700–308,500 100% additional benefit not proven
Remdesivir (1) Veklury® Gilead Sciences GmbH Infectious diseases COVID-19, ≥ 12 years, additional oxygen supply 45,000–79,000 34% Hint for minor additional benefit
Avatrombopag (1) Doptelet® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Thrombocytopenia in chronic liver disease 1,790–24,130 100% additional benefit not proven
Avatrombopag (2) Doptelet® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Immune thrombocytopenia (ITP) 4,260–10,830 100% additional benefit not proven
Fostemsavir (1) Rukobia® ViiV Healthcare GmbH Infectious diseases Multidrug-resistant HIV infection 80–240 100% additional benefit not proven
Pembrolizumab (14) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Hodgkin lymphoma (HL), pre-treated patients, ≥ 3 years 120–240 46% Hint for considerable additional benefit
Pembrolizumab (16) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Urothelial carcinoma (UC), CPS ≥ 10, first-line 225–380 100% additional benefit not proven
Pembrolizumab (15) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Colorectal carcinoma (CRC) with MSI-H or dMMR, first-line 370–955 87% Hint for minor additional benefit
Bedaquilin (4) Sirturo® Janssen-Cilag GmbH Infectious diseases Multidrug-resistant pulmonary tuberculosis, 5 to 11 years 1 100% Hint for non-quantifiable additional benefit Orphan
Imlifidase (1) Idefirix® Hansa Biopharma AB Genitourinary system diseases Desensitisation in kidney transplantation 3–69 100% Hint for non-quantifiable additional benefit Orphan
Fedratinib (1) Inrebic® Celgene GmbH Oncological diseases Myelofibrosis (MF) 740–3,590
1,370–5,280 		100% Hint for non-quantifiable additional benefit Orphan repealed subpopulations
Tucatinib (1) Tukysa® Seagen Germany GmbH Oncological diseases Breast cancer (BC) HER2+, at least 2 previous therapies, combination with trastuzumab and capecitabine 1,350–1,640 100% Hint for considerable additional benefit
Selpercatinib (3) Retsevmo® Lilly Deutschland GmbH Oncological diseases Thyroid carcinoma (MTC), RET fusion+, after sorafenib and/or lenvatinib pre-therapy 2–16 100% additional benefit not proven
Selpercatinib (2) Retsevmo® Lilly Deutschland GmbH Oncological diseases Medullary thyroid carcinoma (MTC), RET-mutated, after cabozantinib and/or vandetanib prior therapy, ≥ 12 years 5–80 100% additional benefit not proven
Selpercatinib (1) Retsevmo® Lilly Deutschland GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), RET fusion+, after platinum-based chemotherapy and/or immunotherapy 55–200 100% additional benefit not proven
Avelumab (4) Bavencio® Merck Serono GmbH und Pfizer Pharma GmbH Oncological diseases Urothelial carcinoma (UC), first-line 4,125 100% Hint for considerable additional benefit
Esketamin (1) Spravato® Janssen-Cilag GmbH Mental illnesses Depression, therapy resistant, combination with SSRI or SNRI 0
932,000–974,000 		100% additional benefit not proven repealed
Esketamin (2) Spravato® Janssen-Cilag GmbH Mental illnesses Depression, acute treatment, combination therapy 49,100–69,200 100% Hint for minor additional benefit
Beclometason / Formoterol / Glycopyrronium (1) Trimbow® Chiesi GmbH Respiratory system diseases Asthma 0
290,000 		100% additional benefit not proven repealed
Baloxavir marboxil (2) Xofluza® Roche Pharma AG Infectious diseases Influenza prophylaxis, ≥ 12 years 1,909,000–3,965,000 58% Indication of considerable additional benefit
Baloxavir marboxil (1) Xofluza® Roche Pharma AG Infectious diseases Influenza, ≥ 12 years 1,871,000–3,303,000 100% additional benefit not proven
Brexucabtagen-Autoleucel / Autologe Anti-CD19-transduzierte CD3+ Zellen (1) Tecartus® Gilead Sciences GmbH Oncological diseases Mantle cell lymphoma (MCL), pretreated 105–150 100% Hint for non-quantifiable additional benefit Orphan
Acalabrutinib (3) Calquence® AstraZeneca GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), at least 1 pretreatment 2,020–7,540 40% Hint for considerable additional benefit
Blinatumomab (5) Blincyto® Amgen GmbH Oncological diseases B-cell acute lymphoblastic leukaemia, relapsed or refractory, Ph+ CD19+ 5–10 100% Hint for non-quantifiable additional benefit Orphan
Pertuzumab / Trastuzumab (1) Phesgo® Roche Pharma AG Oncological diseases Breast cancer (BC) HER2+, metastatic or locally recurrent 2,470–4,000 100% additional benefit not proven
Pertuzumab / Trastuzumab (2) Phesgo® Roche Pharma AG Oncological diseases Breast cancer (BC) HER2+, locally advanced or inflammatory or early with high risk of recurrence, neoadjuvant 2,690–3,450 100% additional benefit not proven
Pertuzumab / Trastuzumab (3) Phesgo® Roche Pharma AG Oncological diseases Breast cancer (BC) early stage, HER2+, adjuvant treatment 0
1,970–3,200 		100% Hint for minor additional benefit repealed
Levofloxacin / Dexamethason (1) Ducressa® Santen GmbH Eye diseases Infections and inflammations associated with cataract surgery 454,000–811,000 100% additional benefit not proven
Dolutegravir (3) Tivicay® ViiV Healthcare GmbH Infectious diseases HIV-Infection, children ≥ 4 weeks to < 6 years 29 100% additional benefit not proven
Niraparib (4) Zejula® GlaxoSmithKline GmbH & Co. KG Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma 700–1,000 100% additional benefit not proven Orphan (turnover limit)
Fenfluramin (1) Fintepla® Zogenix GmbH Nervous system diseases Dravet syndrome, ≥ 2 years 450–2,450 100% Hint for considerable additional benefit Orphan
Inclisiran (1) Leqvio® Novartis Pharma GmbH Metabolic diseases Primary hypercholesterolaemia or mixed dyslipidaemia 284,750–410,900 100% additional benefit not proven
Upadacitinib (2) Rinvoq® AbbVie Deutschland GmbH & Co. KG Skin diseases Psoriasis arthritis (PA) 29,100 69% Hint for considerable additional benefit
Upadacitinib (3) Rinvoq® AbbVie Deutschland GmbH & Co. KG Musculoskeletal system diseases Ankylosing spondylitis 7,800–16,800 100% additional benefit not proven
Carfilzomib (4) Kyprolis® Amgen GmbH Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with daratumumab and dexamethasone 4,700–7,000 100% additional benefit not proven Orphan (turnover limit)
Belimumab (3) Benlysta® GlaxoSmithKline GmbH & Co. KG Musculoskeletal system diseases Lupus nephritis n.d. discontinued
Nivolumab (14) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Squamous cell carcinoma of esophagus, pretreated patients 740–2,060 36% Hint for minor additional benefit
Afamelanotid (2) Scenesse® Clinuvel (Europe) Limited Metabolic diseases Erythropoietic protoporphyria 540–1,090 100% Hint for non-quantifiable additional benefit Orphan
Dupilumab (5) Dupixent® Sanofi-Aventis Deutschland GmbH Skin diseases Atopic dermatitis (AD), 6 to 11 years 9,700–14,100 100% Hint for non-quantifiable additional benefit
Lenvatinib (Kisplyx, 2) Kisplyx® Eisai GmbH Oncological diseases Renal cell carcinoma (RCC) 1,770–3,530 100% additional benefit not proven
Lumasiran (1) Oxlumo® Alnylam Germany GmbH Metabolic diseases Hyperoxaluria 50–880 100% Hint for non-quantifiable additional benefit Orphan
Ipilimumab (6) Yervoy® Bristol-Myers Squibb Pharma EEIG Oncological diseases Non-small cell lung carcinoma (NSCLC), first line, combination with nivolumab and platin-based chemotherapy 14,340–16,180 73% Indication of minor additional benefit
Nivolumab (13) Opdivo® Bristol-Myers Squibb Pharma EEIG Oncological diseases Non-small cell lung carcinoma (NSCLC), combination with ipilimumab and platinum-based chemotherapy, first-line 14,340–16,180 73% Indication of minor additional benefit
Perampanel (4) Fycompa® Eisai GmbH Nervous system diseases Epilepsy, focal seizures, ≥ 12 years, ≥ 4 years 2,950–6,700 100% additional benefit not proven
Perampanel (5) Fycompa® Eisai GmbH Nervous system diseases Epilepsy, primary generalised seizures, ≥ 12 years, ≥ 7 years to < 12 years 195–663 100% additional benefit not proven
Sucroferric Oxyhydroxide (2) Velphoro® Fresenius Medical Care Deutschland GmbH Other diseases Serum phosphate level control in chronic kidney disease, ≥ 2 to 18 years 390–470 100% additional benefit not proven
Olaparib (5) Lynparza® AstraZeneca GmbH Oncological diseases Pancreatic adenocarcinoma (AC), BRCA1/2 mutations, maintenance therapy 75–95 100% additional benefit not proven
Olaparib (6) Lynparza® AstraZeneca GmbH Oncological diseases Prostate carcinoma (PC), BRCA1/2 mutations, progression after hormonal treatment 2,290–3,040 100% Hint for considerable additional benefit
Olaparib (7) Lynparza® AstraZeneca GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, FIGO stages III and IV, HRD-positive, maintenance therapy, combination with bevacizumab 0
1,030 		100% additional benefit not proven repealed
Sebelipase alfa (2) Kanuma® Alexion Pharma Germany GmbH Metabolic diseases Lysosomal acid lipase deficiency 35–70 100% Hint for non-quantifiable additional benefit Orphan
Acalabrutinib (2) Calquence® AstraZeneca GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL); combination with obinutuzumab, first-line 2,880–3,780 25% Hint for minor additional benefit
Acalabrutinib (1) Calquence® AstraZeneca GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL); monotherapy, first-line 2,880–3,780 25% Hint for minor additional benefit
Tezacaftor / Ivacaftor (5) Symkevi® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with ivacaftor in patients 6 to < 12 years (heterozygous for F508del and RF mutation) 50 100% additional benefit not proven Orphan (turnover limit)
Guselkumab (2) Tremfya® Janssen-Cilag GmbH Skin diseases Psoriatic arthritis (PA) 29,100 100% additional benefit not proven
Nusinersen (2) Spinraza® Biogen GmbH Nervous system diseases Spinal muscular atrophy (SMA) 1,013–1,203 19% Indication of major additional benefit Orphan (turnover limit)
Tezacaftor / Ivacaftor (4) Symkevi® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with ivacaftor in patients 6 to < 12 years (homozygous for F508del) 470 100% additional benefit not proven Orphan (turnover limit)
Tafamidis (4) Vyndaqel® Pfizer Pharma GmbH Metabolic diseases Amyloid cardiomyopathy 1,760–1,810 100% Indication of considerable additional benefit Orphan (turnover limit)
Tafamidis (3) Vyndaqel® Pfizer Pharma GmbH Metabolic diseases Amyloid Neuropathy 230 100% additional benefit not proven Orphan (turnover limit)
Niraparib (3) Zejula® GlaxoSmithKline GmbH & Co. KG Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, FIGO stages III and IV, maintenance therapy 2,010–2,810 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (19) Kalydeco® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with tezacaftor/ivacaftor in patients 6 to < 12 years (heterozygous for F508del and MF mutation) 50 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (18) Kalydeco® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with tezacaftor/ivacaftor in patients 6 to < 12 years (homozygous for F508del mutation) 470 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (17) Kalydeco® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), patients from 4 to < 6 months, R117H mutation 1 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor (16) Kalydeco® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), patients from 4 to < 6 months, gating mutations 1 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Dapagliflozin (5) Forxiga® AstraZeneca GmbH · Cardiovascular diseases Chronic heart failure (CHF) 2,061,700–2,273,000 100% Hint for considerable additional benefit
Crizanlizumab (1) Adakveo® Novartis Pharma GmbH Hematopoietic diseases Prevention of recurrent vasoocclusive crises in sickle cell anaemia, ≥16 years 0
390–1,690 		100% Hint for minor additional benefit Orphan repealed
Amikacin (liposomal) (1) Arikayce liposomal® Insmed Germany GmbH Infectious diseases Mycobacterium avium complex (MAC) lung infections 350–760 100% Hint for non-quantifiable additional benefit Orphan
Atezolizumab (8) Tecentriq® Roche Pharma AG Oncological diseases Hepatocellular carcinoma (HCC), combination with bevacizumab 1,710–4,970 76% Indication of considerable additional benefit
Eszopiclon (1) Lunivia® Hennig Arzneimittel GmbH & Co. KG Nervous system diseases Sleep disorders n.d. 100% additional benefit not proven
Baricitinib (2) Olumiant® Lilly Deutschland GmbH Skin diseases Atopic dermatitis (AD) 52,000 100% additional benefit not proven
Ixazomib (2) Ninlaro® Takeda GmbH Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with lenalidomide and dexamethasone 4,700–7,000 100% Hint for non-quantifiable additional benefit Orphan
Avapritinib (1) Ayvakyt® Blueprint Medicines (Germany) GmbH Oncological diseases Gastrointestinal stromal tumor (GIST) 0
1–90 		100% Hint for non-quantifiable additional benefit Orphan repealed
Burosumab (3) Crysvita® Kyowa Kirin GmbH Metabolic diseases X-linked hypophosphatemia (XLH), ≥ 18 years 0
410–810 		100% Hint for minor additional benefit Orphan repealed
Bempedoinsäure / Ezetimib (1) Nustendi® Daiichi Sankyo Deutschland GmbH Metabolic diseases Primary hypercholesterolaemia or mixed dyslipidaemia 284,750–286,750 100% additional benefit not proven
Bempedoinsäure (1) Nilemdo® Daiichi Sankyo Deutschland GmbH Metabolic diseases Primary hypercholesterolaemia or mixed dyslipidaemia 284,750–286,750 100% additional benefit not proven
Semaglutid (Rybelsus, Ozempic, 1) Rybelsus, Ozempic® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 2 0
2,107,999–2,108,001 		100% additional benefit not proven repealed
Cannabidiol (4) Epidyolex® GW Pharmaceuticals plc Nervous system diseases Lennox-Gastaut syndrome, ≥ 2 years, combination with clobazam 0
2,600–22,700 		100% Hint for considerable additional benefit Orphan repealed
Cannabidiol (3) Epidyolex® GW Pharmaceuticals plc Nervous system diseases Dravet syndrome, ≥ 2 years, combination with clobazam 0
1,100–3,100 		100% Hint for considerable additional benefit Orphan repealed
Filgotinib (1) Jyseleca® Gilead Sciences GmbH Musculoskeletal system diseases Rheumatoid arthritis (RA) 89,710–193,750 33% Hint for minor additional benefit
Ibrutinib (8) Imbruvica® Janssen-Cilag GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), first-line, combination with rituximab 3,090 59% Hint for considerable additional benefit Orphan (turnover limit)
Durvalumab (2) Imfinzi® AstraZeneca GmbH Oncological diseases Small cell lung cancer (SCLC), first-line, combination with etoposide and either carboplatin or cisplatin 3,210–6,130 100% Hint for minor additional benefit
Sofosbuvir / Velpatasvir (2) Epclusa® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C, ≥ 6 to < 18 years 120 100% additional benefit not proven
Belantamab-Mafodotin (1) Blenrep® GlaxoSmithKline GmbH & Co. KG Oncological diseases Multiple myeloma (MM), at least 4 prior therapies, monotherapy 0
570–1,130 		100% Hint for non-quantifiable additional benefit Orphan repealed
Alpelisib (1) Piqray® Novartis Pharma GmbH Oncological diseases Breast cancer (BC) with PIK3CA mutation, pre-treated patients, combination with fulvestrant 2,321–21,586 41% additional benefit not proven
Bulevirtid (1) Hepcludex® MYR GmbH Infectious diseases Chronic hepatitis Delta 0
300–4,800 		100% Hint for non-quantifiable additional benefit Orphan repealed
Ivacaftor / Tezacaftor / Elexacaftor (2) Kaftrio® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥ 12 years (homozygous for F508del mutation) 2,400 100% Indication of major additional benefit Orphan (turnover limit)
Ivacaftor / Tezacaftor / Elexacaftor (1) Kaftrio® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥12 years (heterozygous for F508del and MF mutation) 1,000 100% Hint for major additional benefit Orphan (turnover limit)
Ivacaftor (14) Kalydeco® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with ivacaftor/tezacaftor/elexacaftor in patients ≥ 12 years (heterozygous for F508del and MF mutation) 1,000 100% Hint for major additional benefit Orphan (turnover limit)
Ivacaftor (15) Kalydeco® Vertex Pharmaceuticals (Ireland) Limited Metabolic diseases Cystic fibrosis (CF), combination therapy with ivacaftor/tezacaftor/elexacaftor in patients ≥ 12 years (homozygous for F508del mutation) 2,400 100% Indication of major additional benefit Orphan (turnover limit)
Entrectinib (1) Rozlytrek® Roche Pharma AG Oncological diseases Non-small cell lung carcinoma (NSCLC), ROS1+, advanced, first-line 462–1,274 100% additional benefit not proven
Entrectinib (2) Rozlytrek® Roche Pharma AG Oncological diseases Solid tumours, neurotrophic tyrosine receptor kinase (NTRK) gene fusion, histology independent 390–770 100% additional benefit not proven
Ibalizumab (1) Trogarzo® Theratechnologies Europe limited Infectious diseases Multidrug-resistant HIV infection 50–110 100% additional benefit not proven
Secukinumab (5) Cosentyx® Novartis Pharma GmbH Skin diseases Plaque psoriasis (PP), ≥ 6 to < 18 years 270–2,035 100% Hint for minor additional benefit
Secukinumab (6) Cosentyx® Novartis Pharma GmbH Musculoskeletal system diseases Axial spondyloarthritis 19,500 100% additional benefit not proven
Secukinumab (4) Cosentyx® Novartis Pharma GmbH Skin diseases Psoriatic arthritis (PA) 29,100 35% Indication of minor additional benefit
Glasdegib (1) Daurismo® PFIZER PHARMA GmbH Oncological diseases Acute myeloid leukaemia (AML), combination with cytarabine (LDAC) 780–840 100% Hint for considerable additional benefit Orphan
Nintedanib (Ofev, 3) Ofev® Boehringer Ingelheim Pharma GmbH & Co. KG Respiratory system diseases Interstitial pulmonary disease with systemic sclerosis 200–10,700 100% additional benefit not proven
Trifaroten (1) Selgamis® Galderma Laboratorium GmbH Skin diseases Acne vulgaris, ≥ 12 years 887,500–1,950,700 100% additional benefit not proven
Nintedanib (Ofev, 4) Ofev® Boehringer Ingelheim Pharma GmbH & Co. KG Respiratory system diseases Progressive fibrosing interstitial lung diseases 4,500–11,400 100% Indication of minor additional benefit
Ravulizumab (2) Ultomiris® Alexion Pharma Germany GmbH Hematopoietic diseases Atypical hemolytic uremic syndrome (aHUS) 210–700 100% additional benefit not proven
Sofosbuvir (3) Sovaldi® Gilead Sciences Ireland UC Infectious diseases Chronic hepatitis C, 3 to < 12 years 30–50 100% Hint for non-quantifiable additional benefit
Luspatercept (1) Reblozyl® Celgene GmbH Hematopoietic diseases Beta thalassemia 0
170–300 		100% Hint for non-quantifiable additional benefit Orphan repealed
Luspatercept (2) Reblozyl® Celgene GmbH Hematopoietic diseases Myelodysplastic syndrome (MDS) 0
840–1,870 		100% Hint for non-quantifiable additional benefit Orphan repealed
Ledipasvir / Sofosbuvir (3) Harvoni® Gilead Sciences Ireland UC Infectious diseases Chronic hepatitis C, 3 to < 12 years 100–170 50% Hint for non-quantifiable additional benefit
Ixekizumab (4) Taltz® Lilly Deutschland GmbH Skin diseases Plaque psoriasis (PP), ≥ 6 to < 18 years, body weight ≥ 25 kg 270–2,035 100% additional benefit not proven
Ixekizumab (3) Taltz® Lilly Deutschland GmbH Musculoskeletal system diseases Axial spondyloarthritis 36,300 100% additional benefit not proven
Osilodrostat (1) Isturisa® Recordati Rare Diseases Germany GmbH Metabolic diseases Endogenous Cushing syndrome 1,130–1,550 100% Hint for non-quantifiable additional benefit Orphan
Ozanimod (1) Zeposia® Celgene GmbH Nervous system diseases Relapsing-remitting multiple sclerosis (MS) 149,500–166,000 90% Indication of minor additional benefit
Caplacizumab (2) Cablivi® Sanofi-Aventis Deutschland GmbH Hematopoietic diseases Thrombotic thrombocytopenic purpura (aTTP)Thrombotic thrombocytopenic purpura (TTP), 12 to < 18 years 2–3 100% Hint for non-quantifiable additional benefit Orphan
Encorafenib (2) Braftovi® Pierre Fabre Pharma GmbH Oncological diseases Metastatic colorectal carcinoma (CRC) 525–1,235 100% Hint for considerable additional benefit
Fostamatinib (1) Tavlesse® Grifols Deutschland GmbH Hematopoietic diseases Chronic immune thrombocytopenia 4,200–9,700 100% additional benefit not proven
Ivacaftor (13) Kalydeco® Vertex Pharmaceuticals Metabolic diseases Cystic fibrosis (CF), patients ≥ 6 months to < 18 years 26 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Tezacaftor / Ivacaftor (2) Symkevi® Vertex Pharmaceuticals Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥ 12 years (homozygous for F508del) 2,400 100% additional benefit not proven Orphan (turnover limit)
Tezacaftor / Ivacaftor (3) Symkevi® Vertex Pharmaceuticals Metabolic diseases Cystic fibrosis (CF), combination treatment with ivacaftor in patients ≥ 12 years (heterozygous for F508del) 200–300 100% additional benefit not proven Orphan (turnover limit)
Onasemnogen-Abeparvovec (1) Zolgensma® AveXis Nervous system diseases Spinal muscular atrophy (SMA) n.d. suspended Orphan
Brentuximab Vedotin (5) Adcetris® Takeda GmbH Oncological diseases Systemic anaplastic large cell lymphoma; first-line; combination with cyclophosphamide, doxorubicin and prednisone 0
125–127 		100% Hint for minor additional benefit Orphan repealed
Mogamulizumab (1) Poteligeo® Kyowa Kirin GmbH Oncological diseases Mycosis Fungoides; Sézary Syndrome 310–460 100% Hint for non-quantifiable additional benefit Orphan
Talazoparib (1) Talzenna® Pfizer Pharma GmbH Oncological diseases Breast cancer (BC) BRCA1/2 mutation, HER2- 410–1,830 100% Hint for considerable additional benefit
Ponatinib (2) Iclusig® Incyte Biosciences Germany GmbH Oncological diseases Chronic myeloid leukaemia (CML) 500–940 100% Hint for non-quantifiable additional benefit Orphan
Ponatinib (3) Iclusig® Incyte Biosciences Germany GmbH Oncological diseases Acute myeloid leukaemia (AML) 25–195 100% Hint for non-quantifiable additional benefit Orphan
Naldemedin (1) Rizmoic® Hexal AG Digestive system diseases Opioid-induced constipation 65,000 100% additional benefit not proven
Apremilast (2) Otezla® Amgen GmbH Other diseases Behçet's disease 750–2,200 100% additional benefit not proven
Enzalutamid (4) Xtandi® Astellas Pharma GmbH Oncological diseases Prostate carcinoma (PC), non-metastatic, high-risk 1,090–3,800 100% Indication of minor additional benefit
Solriamfetol (1) Sunosi® Jazz Pharmaceuticals Nervous system diseases Narcolepsy 16,200–19,500 100% additional benefit not proven
Brigatinib (2) Alunbrig® Takeda GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, ALK inhibitor-naive patients 420–910 50% Hint for considerable additional benefit
Darolutamid (1) Nubeqa® Bayer Vital GmbH Oncological diseases Prostate carcinoma (PC), non-metastatic, high-risk 1,090–3,800 100% Indication of considerable additional benefit
Venetoclax (4) Venclyxto® AbbVie Deutschland GmbH & Co. KG Oncological diseases Chronic lymphocytic leukaemia (CLL), first-line, in combination with obinutuzumab 3,090–3,099 100% additional benefit not proven
Insulin glargin / Lixisenatid (2) Suliqua® Sanofi-Aventis Deutschland GmbH Metabolic diseases Diabetes mellitus type 2, combination with metformin and with SGLT-2 inhibitors 532,000 100% additional benefit not proven
Givosiran (1) Givlaari® Alnylam Germany GmbH Metabolic diseases Acute hepatic porphyria, ≥ 12 years 1,000–1,700 100% Indication of considerable additional benefit Orphan
Avelumab (3) Bavencio® Merck Serono GmbH Oncological diseases Metastatic Merkel-cell carcinoma (MCC) 370–720 100% additional benefit not proven
Trifluridin / Tipiracil (3) Lonsurf® Servier Deutschland GmbH Oncological diseases Colorectal carcinoma (CRC), pre-treated patients 6,900–12,200 100% Hint for minor additional benefit
Apalutamid (3) Erleada® Janssen-Cilag GmbH Oncological diseases Prostate carcinoma (PC), non-metastatic, high-risk 1,090–3,800 100% Indication of minor additional benefit
Cobicistat (2) Tybost® Gilead Sciences GmbH Infectious diseases HIV infection, combination with atazanavir or darunavir, 12 to < 18 years 130 100% additional benefit not proven
Ceftolozan / Tazobactam (1) Zerbaxa® MSD SHARP & DOHME GMBH Infectious diseases Ospital-acquired pneumonia, ventilator-associated pneumonia n.d. discontinued
Ceftolozan / Tazobactam (2) Zerbaxa® MSD SHARP & DOHME GMBH Infectious diseases Complicated intra-abdominal infections n.d. discontinued
Ceftolozan / Tazobactam (3) Zerbaxa® MSD SHARP & DOHME GMBH Infectious diseases Complicated urinary tract infections n.d. discontinued
Ceftolozan / Tazobactam (4) Zerbaxa® MSD SHARP & DOHME GMBH Infectious diseases Acute pyelonephritis n.d. discontinued
Tisagenlecleucel (3) Kymriah® Novartis Pharma GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL) 0
50–65 		100% Hint for non-quantifiable additional benefit Orphan repealed
Tisagenlecleucel (4) Kymriah® Novartis Pharma GmbH Oncological diseases Diffuse large B-cell lymphoma (DLBCL) 0
450–720 		100% Hint for non-quantifiable additional benefit Orphan repealed
Indacaterol / Mometason (1) Atectura Breezhaler® Novartis Pharma GmbH Respiratory system diseases Bronchial asthma 1,000,000–1,100,000 100% additional benefit not proven
Brolucizumab (1) Beovu® Novartis Pharma GmbH Eye diseases Neovascular age-related macular degeneration 0
85,200–681,400 		100% additional benefit not proven repealed
Fidaxomicin (2) Dificlir® Astellas Pharma GmbH Infectious diseases Clostridioides difficile infection, children and adolescents 350 54% Hint for considerable additional benefit
Romosozumab (1) Evenity® UCB Pharma GmbH Musculoskeletal system diseases Osteoporosis (postmenopausal) 475,000 100% Indication of minor additional benefit
Riociguat (2) Adempas® MSD SHARP & DOHME GMBH Cardiovascular diseases Chronic thromboembolic pulmonary hypertension 920–5,460 100% additional benefit not proven Orphan (turnover limit)
Riociguat (3) Adempas® MSD SHARP & DOHME GMBH Cardiovascular diseases Pulmonary arterial hypertension (PAH) 580–7,850 100% additional benefit not proven Orphan (turnover limit)
Abemaciclib (3) Verzenios® Lilly Deutschland GmbH Oncological diseases Mammary carcinoma, HR+, HER2-, combination with fulvestrant 906–4,118
13,776–63,808 		39% Hint for minor additional benefit repealed subpopulations
Tafamidis (2) Vyndaqel® Pfizer Pharma GmbH Metabolic diseases Amyloid cardiomyopathy 0
1,630–1,730 		100% Hint for considerable additional benefit Orphan repealed
Apalutamid (2) Erleada® Janssen-Cilag GmbH Oncological diseases Prostate carcinoma (PC), hormone-sensitive, combination with androgen deprivation therapy 2,590–3,640 100% additional benefit not proven
Ribociclib (4) Kisqali® Novartis Pharma GmbH Oncological diseases Breast cancer (BC) HR+, HER2-, combination with fulvestrant 13,140–59,690 58% Indication of minor additional benefit
Ribociclib (3) Kisqali® Novartis Pharma GmbH Oncological diseases Breast cancer (BC) HR+, HER2-, combination with aromatase inhibitor 7,400–34,790 100% Hint for minor additional benefit
Polatuzumab Vedotin (1) Polivy® Roche Pharma AG Oncological diseases Diffuse large B-cell lymphoma (DLBCL), combination with bendamustine and rituximab 0
730–1,560 		100% Hint for non-quantifiable additional benefit Orphan repealed
Siponimod (1) Mayzent® Novartis Pharma GmbH Nervous system diseases Secondary progressive multiple sclerosis (MS) 13,200–28,900 100% additional benefit not proven
Bedaquilin (3) Sirturo® Janssen-Cilag GmbH Infectious diseases Multidrug-resistant pulmonary tuberculosis, 12 to < 18 years 9–13 100% Hint for non-quantifiable additional benefit Orphan
Daratumumab (5) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma (MM), newly diagnosed, patients not suitable for autologous stem cell transplantation, combination with lenalidomide and dexamethasone 0
3,479–3,670 		100% Hint for minor additional benefit Orphan (turnover limit) repealed
Daratumumab (6) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma (MM), newly diagnosed, patients suitable for autologous stem cell transplantation, combination with bortezomib, thalidomide and dexamethasone 1,800–1,900 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ramucirumab (6) Cyramza® Lilly Deutschland GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), EGFR mutation, first-line 780–1,810 100% additional benefit not proven
Dulaglutid (2) Trulicity® Lilly Deutschland GmbH Metabolic diseases Diabetes mellitus type 2 2,108,000 31% Hint for minor additional benefit
Upadacitinib (1) Rinvoq® AbbVie Deutschland GmbH Musculoskeletal system diseases Rheumatoid arthritis (RA) 88,709–188,210 34% Hint for considerable additional benefit
Trastuzumab Emtansin (2) Kadcyla® Roche Pharma AG Oncological diseases Breast cancer (BC) early stage, HER2+, adjuvant treatment 1,980 100% Indication of minor additional benefit
Indigocarmin (1) Indigokarmin Serb 40 mg/5 ml® SERB SA Genitourinary system diseases Other diagnostics n.d. exempted
Ivacaftor (12) Kalydeco® Vertex Pharmaceuticals Metabolic diseases Cystic fibrosis (CF), ≥ 6 to < 12 months 2 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Neratinib (1) Nerlynx® Pierre Fabre Pharma Oncological diseases Breast cancer (BC) HR+, HER2+, adjuvant therapy 2,330–4,560 100% Hint for minor additional benefit
Gilteritinib (1) Xospata® Astellas Pharma GmbH Oncological diseases Acute myeloid leukaemia (AML), FLT3 mutation 220–580 100% Hint for considerable additional benefit Orphan
Avelumab (2) Bavencio® Merck Serono GmbH / Pfizer Pharma GmbH Oncological diseases Renal cell carcinoma (RCC), first-line, combination with axitinib 3,500 23% Hint for considerable additional benefit
Dupilumab (4) Dupixent® Sanofi-Aventis Deutschland GmbH Respiratory system diseases Chronic rhinosinusitis with nasal polyps 10,500–12,600 100% Indication of considerable additional benefit
Pembrolizumab (13) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Squamous cell carcinoma head and neck, PD-L1 expression ≥ 1%, first-line, combination with platinum and 5-fluorouracil (5-FU) chemotherapy 4,950–5,370 100% Indication of minor additional benefit
Pembrolizumab (11) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Squamous cell carcinoma head and neck, PD-L1 expression ≥ 1%, first-line, monotherapy 4,955–5,370 100% Hint for considerable additional benefit
Pembrolizumab (12) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Renal cell carcinoma (RCC), first-line, combination with axitinib 3,500 23% Indication of considerable additional benefit
Belimumab (2) Benlysta® GlaxoSmithKline GmbH & Co. KG Musculoskeletal system diseases Systemic lupus erythematosus 30–550 100% Hint for non-quantifiable additional benefit
Betibeglogene Autotemcel (1) Zynteglo® Bluebird bio (Germany) GmbH Hematopoietic diseases Beta thalassemia 50 100% Hint for non-quantifiable additional benefit Orphan
Cannabidiol (1) Epidyolex® GW Pharmaceuticals plc Nervous system diseases Dravet syndrome, ≥ 2 years, combination with clobazam 0
1,100–3,100 		100% Hint for non-quantifiable additional benefit Orphan repealed
Niraparib (2) Zejula® TESARO Bio Germany GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma 0
1,900–2,400 		100% additional benefit not proven Orphan (turnover limit) repealed
Cannabidiol (2) Epidyolex® GW Pharmaceuticals plc Nervous system diseases Lennox-Gastaut syndrome, ≥ 2 years, combination with clobazam 0
2,600–22,700 		100% Hint for non-quantifiable additional benefit Orphan repealed
Larotrectinib (1) Vitrakvi® Bayer Vital GmbH Oncological diseases Solid tumours, neurotrophic tyrosine receptor kinase (NTRK) gene fusion, histology independent 390–770 100% additional benefit not proven
Atezolizumab (5) Tecentriq® Roche Pharma AG Oncological diseases Non-small cell lung carcinoma (NSCLC), non-squamous, 1st line, combination with bevacizumab, paclitaxel and carboplatin; maintenance therapy 8,990–9,590 100% additional benefit not proven
Trifluridin / Tipiracil (2) Lonsurf® Servier Deutschland GmbH Oncological diseases Metastatic gastric cancer, pre-treated patients 590–1,030 100% Indication of minor additional benefit
Asfotase alfa (2) Strensiq® Alexion Pharma Germany GmbH Metabolic diseases Hypophosphatasia (HPP) 1,074 0.8% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Atezolizumab (4) Tecentriq® Roche Pharma AG Oncological diseases Breast cancer (BC), triple-negative, PD-L1 expression ≥1% 920–1,110 100% Hint for non-quantifiable additional benefit
Atezolizumab (6) Tecentriq® Roche Pharma AG Oncological diseases Non-small cell lung carcinoma (NSCLC), non-squamous, 1st line, combination with nab-paclitaxel and carboplatin; maintenance therapy 8,020–9,110 100% additional benefit not proven
Atezolizumab (7) Tecentriq® Roche Pharma AG Oncological diseases Small cell lung cancer (SCLC), first-line, combination with carboplatin and etoposide; maintenance therapy 7,280–8,550 100% Hint for minor additional benefit
Elotuzumab (2) Empliciti® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Multiple myeloma (MM), at least 2 previous therapies, combination with pomalidomide and dexamethasone 0
2,500 		100% Hint for considerable additional benefit repealed
Burosumab (2) Crysvita® Kyowa Kirin GmbH Metabolic diseases X-linked hypophosphatemia (XLH), ≥ 1 to < 18 years 0
200–500 		100% Hint for non-quantifiable additional benefit Orphan repealed
Ropeginterferon alfa-2b (1) Besremi® AOP Orphan Pharmaceuticals AG Oncological diseases Polycythemia vera 1,860–19,800 100% additional benefit not proven
Ivacaftor (11) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), 12 ro < 24 months 5 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor (10) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), patients ≥ 18 years with R117H mutation 35–44 100% Hint for minor additional benefit Orphan (turnover limit)
Ivacaftor (9) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), patients from 2 to 5 years 15 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor (8) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), various gating mutations, ≥ 6 years 10–11 100% Hint for non-quantifiable additional benefit Orphan (turnover limit)
Ivacaftor (7) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), heterozygous for F508del, ≥ 12 years, combination with tezacaftor 200–300 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (6) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), homozygous for F508del, ≥ 12 years, combination with tezacaftor 2,400 100% additional benefit not proven Orphan (turnover limit)
Ivacaftor (5) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), G551D mutation, ≥ 6 years 210 86% Hint for considerable additional benefit Orphan (turnover limit)
Ramucirumab (5) Cyramza® Lilly Deutschland GmbH Oncological diseases Hepatocellular carcinoma (HCC) 500–2,200 100% Proof of minor additional benefit
Dupilumab (2) Dupixent® Sanofi-Aventis Deutschland GmbH Respiratory system diseases Bronchial asthma, ≥ 12 years 17,560–54,300 100% additional benefit not proven
Dupilumab (3) Dupixent® Sanofi-Aventis Deutschland GmbH Skin diseases Atopic dermatitis (AD), 12 to < 18 years 5,300–10,600 100% Hint for non-quantifiable additional benefit
Andexanet alfa (1) Ondexxya® Portola Deutschland GmbH Hematopoietic diseases Antidote for reversal of anticoagulation (factor Xa) 0
4,200–27,600 		100% additional benefit not proven repealed
Ibrutinib (6) Imbruvica® Janssen-Cilag GmbH Oncological diseases Chronische lymphatische Leukämie (CLL), Erstlinie, Kombination mit Obinutuzumab 3,090 26% Hint for minor additional benefit Orphan (turnover limit)
Ibrutinib (7) Imbruvica® Janssen-Cilag GmbH Oncological diseases Waldenström's disease, combination with rituximab 590–1,180 100% additional benefit not proven Orphan (turnover limit)
Volanesorsen (1) Waylivra® Akcea Therapeutics Germany GmbH Metabolic diseases Chylomicronaemia syndrome 60–120 100% Hint for non-quantifiable additional benefit Orphan
Turoctocog alfa pegol (1) Esperoct® Novo Nordisk Pharma GmbH Hematopoietic diseases Hemophilia A 2,840–3,190 100% additional benefit not proven
Dolutegravir / Lamivudin (1) Dovato® ViiV Healthcare GmbH Infectious diseases HIV infection, ≥ 12 years 52,060–61,380 100% additional benefit not proven
Ravulizumab (1) Ultomiris® Alexion Pharma Germany GmbH Hematopoietic diseases Paroxysmal hemoglobinuria 260–700 100% additional benefit not proven
Cemiplimab (1) Libtayo® Sanofi-Aventis Deutschland GmbH Oncological diseases Squamous cell carcinoma (SCC) 450–1,400 100% additional benefit not proven
Indacaterolacetat / Glycopyrroniumbromid / Mometasonfuroat (1) Enerzair Breezhaler® Novartis Pharma GmbH Respiratory system diseases Bronchial asthma 100,000 100% additional benefit not proven
Olaparib (3) Lynparza® AstraZeneca GmbH Oncological diseases Breast cancer (BC) BRCA1/2 mutations, HER2- 460–710 100% Hint for minor additional benefit
Olaparib (4) Lynparza® AstraZeneca GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, BRCA-mutated, FIGO stages III and IV, maintenance therapy 0
70–325 		100% additional benefit not proven repealed
Vigabatrin (2) Kigabeq® Desitin Arzneimittel GmbH Nervous system diseases Epilepsy, West syndrome, < 7 years 0
1,000–2,400 		100% additional benefit not proven repealed
Vigabatrin (1) Kigabeq® Desitin Arzneimittel GmbH Nervous system diseases Epilepsy, focal seizures, < 7 years 0
7,300–14,500 		100% additional benefit not proven repealed
Pegvaliase (1) Palynziq® BioMarin International Limited Metabolic diseases Phenylketonuria 435 100% Hint for non-quantifiable additional benefit Orphan
Dapagliflozin / Metformin (3) Xigduo® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2 1,139,000 50% Hint for minor additional benefit
Dapagliflozin (4) Forxiga® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2 2,108,000 41% Hint for minor additional benefit
Pomalidomid (3) Imnovid® Celgene GmbH Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with bortezomib and dexamethasone 3,060–3,450 100% additional benefit not proven Orphan (turnover limit)
Empagliflozin / Linagliptin (1) Glyxambi® Boehringer Ingelheim Pharma GmbH & Co. KG Metabolic diseases Diabetes mellitus type 2 326,100–341,100 100% additional benefit not proven
Risankizumab (1) Skyrizi® AbbVie Deutschland GmbH & Co. KG Skin diseases Plaque psoriasis (PP) 35,900–121,500 82% Proof of considerable additional benefit
Lorlatinib (1) Lorviqua® Pfizer Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, pre-treated 200–1,310 100% additional benefit not proven
Fremanezumab (1) Ajovy® Teva GmbH Nervous system diseases Migraine prophylaxis 1,443,400–1,471,000 1.0% Hint for considerable additional benefit
Prasteron (1) Intrarosa® Endoceutics, Inc. Genitourinary system diseases Vulvovaginal atrophy n.d. discontinued
Dacomitinib (1) Vizimpro® Pfizer Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), EGFR mutation, first-line 890–2,210 100% additional benefit not proven
Lisdexamfetamindimesilat (Elvanse Adult, 1) Elvanse Adult® Shire Deutschland GmbH, jetzt Teil der Takeda Group Mental illnesses Attention deficit hyperactivity disorder (ADHD), ≥ 18 years 73,000 100% additional benefit not proven
Dapagliflozin (3) Forxiga® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 1 0
19,200 		100% Hint for minor additional benefit repealed
Radium-223-dichlorid (2) Xofigo® Bayer Vital GmbH Oncological diseases Prostate carcinoma (PC) 3,810–4,560 100% additional benefit not proven
Voretigen Neparvovec (1) Luxturna® Novartis Pharma GmbH Eye diseases Hereditary retinal dystrophy 0
100–530 		100% Hint for considerable additional benefit Orphan repealed
Glecaprevir / Pibrentasvir (2) Maviret® AbbVie Deutschland GmbH & Co. KG Infectious diseases Chronic hepatitis C, 12 to < 18 years 540 100% additional benefit not proven
Nintedanib (Ofev, 2) Ofev® Boehringer Ingelheim Pharma GmbH & Co. KG Respiratory system diseases Idiopathic pulmonary fibrosis 1,800–18,900 100% Hint for considerable additional benefit Orphan (turnover limit)
Pembrolizumab (10) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Non-small cell lung carcinoma (NSCLC), squamous cell histology, first-line, combination with carboplatin and (nab-) paclitaxel 5,340–5,570 71% Hint for considerable additional benefit
Pembrolizumab (9) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Non-small cell lung carcinoma (NSCLC), non-squamous cell histology, first-line, combination with pemetrexed and platinum chemotherapy 8,020–9,120 100% Hint for non-quantifiable additional benefit
Pembrolizumab (8) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Melanoma, adjuvant therapy 2,670–3,400 100% Indication of non-quantifiable additional benefit
Galcanezumab (1) Emgality® Lilly Deutschland GmbH Nervous system diseases Migraine prophylaxis 1,443,400–1,471,000 1.0% Hint for considerable additional benefit
Brentuximab Vedotin (4) Adcetris® Takeda GmbH & Co. KG Oncological diseases Hodgkin lymphoma (HL), CD30+, first-line 220–380 100% non-quantifiable additional benefit Orphan
Emicizumab (2) Hemlibra® Roche Pharma AG Hematopoietic diseases Hemophilia A, without factor VIII inhibitors 2,000 100% additional benefit not proven
Regadenoson (2) Rapiscan® GE Healthcare Buchler GmbH & Co. KG Other diseases Stenosis, Fractional Flow Reserve Measurement (FFR) 0
780,000 		100% additional benefit not proven repealed
Rucaparib (1) Rubraca® Clovis Oncology Germany GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, BRCA-mutated, after at least 2 previous therapies 0
95 		100% additional benefit not proven repealed
Rucaparib (2) Rubraca® Clovis Oncology Germany GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, maintenance therapy 0
1,900–2,400 		100% additional benefit not proven repealed
Blinatumomab (4) Blincyto® Amgen GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL), MRD-positive patients 40–110 100% non-quantifiable additional benefit Orphan
Lumacaftor / Ivacaftor (3) Orkambi® Vertex Pharmaceuticals (Europe) Limited Metabolic diseases Cystic fibrosis (CF), homozygous F508del mutation in the CFTR gene, 2 to 5 years 0
280 		100% Hint for non-quantifiable additional benefit repealed
Blinatumomab (3) Blincyto® Amgen GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL), ≥ 1 to <18 years 30–80 100% non-quantifiable additional benefit Orphan
Lenvatinib (3) Lenvima® Eisai GmbH Oncological diseases Thyroid carcinoma (DTC) 740–770 100% additional benefit not proven
Ipilimumab (5) Yervoy® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Renal cell carcinoma (RCC), first-line, combination with nivolumab 2,110–2,850 100% Indication of considerable additional benefit
Nivolumab (12) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Renal cell carcinoma (RCC), first-line, combination with ipilimumab 2,110–2,850 100% Indication of considerable additional benefit
Mexiletin (1) Namuscla® Lupin Europe GmbH Nervous system diseases Myotonia 530–650 100% non-quantifiable additional benefit Orphan
Apalutamid (1) Erleada® Janssen-Cilag GmbH Oncological diseases Prostate carcinoma (PC), non-metastatic, high-risk 0
810–1,180 		100% Hint for minor additional benefit repealed
Lanadelumab (1) Takhzyro® Shire Deutschland GmbH, Teil der Takeda Group Other diseases Hereditary angioedema (HAE) 0
140–430 		100% considerable additional benefit Orphan repealed
Doravirin (1) Pifeltro® MSD SHARP & DOHME GMBH Infectious diseases HIV infection 57,800–73,700 100% additional benefit not proven
Doravirin / Lamivudin / Tenofovirdisoproxil (1) Delstrigo® MSD SHARP & DOHME GMBH Infectious diseases HIV infection 48,800–68,000 100% additional benefit not proven
Glycerolphenylbutyrat (2) Ravicti® Swedish Orphan Biovitrum GmbH Metabolic diseases Urea cycle disorders (UCD), 0 to < 2 months 10–18 100% non-quantifiable additional benefit Orphan
Brigatinib (1) Alunbrig® Takeda GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, pre-treated with crizotinib 160–1,060 100% additional benefit not proven
Bedaquilin (2) Sirturo® Janssen-Cilag GmbH Infectious diseases Multidrug-resistant pulmonary tuberculosis 0
70–100 		100% considerable additional benefit Orphan repealed
Melatonin (1) Slenyto® InfectoPharm Arzneimittel und Consilium GmbH Nervous system diseases Sleep disorders 0
8,000–86,000 		100% Hint for minor additional benefit repealed
Ribociclib (2) Kisqali® Novartis Pharma GmbH Oncological diseases Breast cancer (BC) HR+, HER2-, postmenopausal, premenopausal and perimenopausal women, combination with fulvestrant, combination with aromatase inhibitor 9,450–45,780
21,940–106,310 		100% additional benefit not proven repealed subpopulations
Damoctocog alfa pegol (1) Jivi® Bayer Vital GmbH Hematopoietic diseases Hemophilia A, ≥ 12 years 2,840–3,190 100% additional benefit not proven
Pembrolizumab (7) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Urothelial carcinoma (UC), CPS ≥ 10, first-line 0
240–420 		100% additional benefit not proven repealed
Fingolimod (5) Gilenya® Novartis Pharma GmbH Nervous system diseases Multiple sclerosis (MS), ≥ 10 to < 18 years 190–840 57% Hint for non-quantifiable additional benefit
Atezolizumab (3) Tecentriq® Roche Pharma AG Oncological diseases Urothelial carcinoma (UC), PD-L1 expression ≥ 5%, first-line 220–380 100% additional benefit not proven
Cabozantinib (Cabometyx, 4) Cabometyx® Ipsen Pharma GmbH Oncological diseases Hepatocellular carcinoma (HCC) 1,280–4,900 100% Hint for minor additional benefit
Tezacaftor / Ivacaftor (1) Symkevi® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), F508del mutation, ≥ 12 years 0
2,600–2,700 		91% considerable additional benefit Orphan repealed
Enzalutamid (3) Xtandi® Astellas Pharma GmbH Oncological diseases Prostate carcinoma (PC), non-metastatic, high-risk 0
810–1,180 		100% additional benefit not proven repealed
Venetoclax (2) Venclyxto® AbbVie Deutschland GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), combination with rituximab 2,000–7,200 37% Indication of minor additional benefit
Venetoclax (3) Venclyxto® AbbVie Deutschland GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), monotherapy 300–700 100% additional benefit not proven
Insulin degludec (4) Tresiba® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 2 776,100–991,100 100% additional benefit not proven
Fluticasonfuroat / Umeclidinium / Vilanterol (Trelegy Ellipta, Elebrato Ellipta, 1) Trelegy Ellipta, Elebrato Ellipta® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Chronic obstructive pulmonary disease (COPD), inadequate control with LAMA and LABA 524,000–1,217,000 100% additional benefit not proven
Tildrakizumab (1) Ilumetri® Almirall Hermal GmbH Skin diseases Plaque psoriasis (PP) 157,100–234,400 100% additional benefit not proven
Alirocumab (2) Praluent® Sanofi-Aventis Deutschland GmbH Metabolic diseases Hypercholesterolemia or mixed dyslipidaemia 271,750 100% additional benefit not proven
Axicabtagen-Ciloleucel (2) Yescarta® Kite, a Gilead Company Oncological diseases Primary mediastinal large B-cell lymphoma (PMBCL) 0
5–9 		100% non-quantifiable additional benefit Orphan repealed
Axicabtagen-Ciloleucel (1) Yescarta® Kite, a Gilead Company Oncological diseases Diffuse large B-cell lymphoma (DLBCL) 0
440–700 		100% non-quantifiable additional benefit Orphan repealed
Abemaciclib (2) Verzenios® Lilly Deutschland GmbH Oncological diseases Breast cancer (BC), HR+, HER2-, combination with fulvestrant 14,560–70,550 100% additional benefit not proven
Abemaciclib (1) Verzenios® Lilly Deutschland GmbH Oncological diseases Breast cancer (BC), HR+, HER2-, combination with aromatase inhibitor 14,560–70,550 100% additional benefit not proven
Semaglutid (1) Ozempic® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 2 0
1,969,100–2,184,100 		37% Hint for minor additional benefit repealed
Erenumab (1) Aimovig® Novartis Pharma GmbH Nervous system diseases Migraine prophylaxis 15,400–26,000
1,443,400–1,471,000 		1.0% Hint for considerable additional benefit repealed subpopulations
Pembrolizumab (6) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Squamous cell carcinoma head and neck 470–3,290 100% additional benefit not proven
Ocriplasmin (2) Jetrea® Oxurion NV Eye diseases Vitreomacular traction (VMT) 1,160–16,500 94% Hint for minor additional benefit
Durvalumab (1) Imfinzi® AstraZeneca GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), maintenance therapy 1,600–1,800 100% Hint for considerable additional benefit
Ivacaftor (4) Kalydeco® Vertex Pharmaceuticals (Germany) GmbH Metabolic diseases Cystic fibrosis (CF), ≥ 12 years, combination with tezacaftor n.d. discontinued Orphan
Trametinib (3) Mekinist® Novartis Pharma GmbH Oncological diseases Melanoma, in combination with dabrafenib, BRAF V600 mutation, adjuvant therapy 1,290–1,590 100% Indication of considerable additional benefit
Dabrafenib (4) Tafinlar® Novartis Pharma GmbH Oncological diseases Melanoma, BRAF V600 mutation, combination with trametinib, adjuvant therapy 1,290–1,590 100% Indication of considerable additional benefit
Patisiran (1) Onpattro® Alnylam Germany GmbH Metabolic diseases Amyloidosis 0
350 		100% considerable additional benefit Orphan repealed
Inotersen (1) Tegsedi® Akcea Therapeutics Germany GmbH Metabolic diseases Amyloidosis 350 100% non-quantifiable additional benefit Orphan
Metreleptin (1) Myalepta® Aegerion Pharmaceuticals GmbH Metabolic diseases Lipodystrophy 90–180 100% non-quantifiable additional benefit Orphan
Binimetinib (1) Mektovi® Pierre Fabre Pharma GmbH Oncological diseases Melanoma, BRAF V600 mutation, combination with encorafenib 1,390 100% additional benefit not proven
Encorafenib (1) Braftovi® Pierre Fabre Pharma GmbH Oncological diseases Melanoma, BRAF V600 mutation, combination with binimetinib 1,390 100% additional benefit not proven
Palbociclib (2) Ibrance® Pfizer Pharma GmbH Oncological diseases Breast cancer (BC), patient population b1 and b2 6,190–30,000 100% additional benefit not proven
Lenvatinib (2) Lenvima® Eisai GmbH Oncological diseases Hepatocellular carcinoma (HCC) 2,630–4,750 100% additional benefit not proven
Daunorubicin / Cytarabin (1) Vyxeos® Jazz Pharmaceuticals Oncological diseases Therapy-related acute myeloid leukaemia (AML); acute myeloid leukaemia (AML) with multilinear dysplasia 310–510 100% considerable additional benefit Orphan
Mepolizumab (2) Nucala® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Bronchial asthma, ≥ 6 to < 18 years 2,200–3,500 100% additional benefit not proven
Sitagliptin (3) Januvia, Xelevia® MSD SHARP & DOHME GMBH Metabolic diseases Diabetes mellitus type 2, combination with metformin 781,600 100% Hint for minor additional benefit
Tenofoviralafenamid (2) Vemlidy® Gilead Sciences GmbH Infectious diseases Chronic hepatitis B, ≥ 12 years 12,400–38,100 100% additional benefit not proven
Caplacizumab (1) Cablivi® Sanofi-Aventis Deutschland GmbH Hematopoietic diseases Thrombotic thrombocytopenic purpura (TTP) 150 100% non-quantifiable additional benefit Orphan
Vestronidase alfa (1) Mepsevii® Ultragenyx Germany GmbH Metabolic diseases Mucopolysaccharidosis (MPS VII; Sly syndrome) 2–7 100% non-quantifiable additional benefit Orphan
Daratumumab (4) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma (MM), first-line, unsuitable for stem cell transplantation, combination with bortezomib, melphalan and prednisone 0
3,380–3,900 		100% Hint for considerable additional benefit Orphan (turnover limit) repealed
Tisagenlecleucel (1) Kymriah® Novartis Pharma GmbH Oncological diseases Diffuse large B-cell lymphoma (DLBCL) 0
440–700 		100% non-quantifiable additional benefit Orphan repealed
Tisagenlecleucel (2) Kymriah® Novartis Pharma GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL) 0
50–65 		100% non-quantifiable additional benefit Orphan repealed
Tofacitinib (3) Xeljanz® Pfizer Pharma GmbH Skin diseases Psoriatic arthritis (PA) 25,900 69% Hint for minor additional benefit
Tofacitinib (4) Xeljanz® Pfizer Pharma GmbH Digestive system diseases Ulcerative colitis 5,300–25,000 100% additional benefit not proven
Nivolumab (11) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, adjuvant therapy 0
2,980–3,780 		100% Hint for non-quantifiable additional benefit repealed
Gemtuzumab Ozogamicin (1) Mylotarg® Pfizer Pharma GmbH Oncological diseases Acute myeloid leukaemia (AML) 560–1,150 100% non-quantifiable additional benefit Orphan
Bosutinib (3) Bosulif® Pfizer Pharma GmbH Oncological diseases Chronic myeloid leukaemia (CML), Ph+ 545–555 100% additional benefit not proven
Ingenolmebutat (2) Picato® Leo Pharma GmbH Skin diseases Actinic keratosis (AK) 0
990,000–1,114,000 		50% Hint for non-quantifiable additional benefit repealed
Brivaracetam (2) Briviact® UCB Pharma GmbH Nervous system diseases Epilepsy, focal seizures, add-on therapy, 4 to < 16 years 5,000–10,000 100% additional benefit not proven
Osimertinib (3) Tagrisso® AstraZeneca GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), first-line 860–2,030 84% Hint for considerable additional benefit
Ipilimumab (4) Yervoy® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, combination with nivolumab 2,500–4,500 85% additional benefit not proven
Nivolumab (10) Opdivo® Bristol-Myers-Squibb GmbH & Co. KGaA Oncological diseases Melanoma, BRAF V600 wild-type, first-line, combination with ipilimumab 270–810 100% Indication of less benefit
Pertuzumab (3) Perjeta® Roche Pharma AG Oncological diseases Breast cancer (BC), early with high risk of recurrence, adjuvant, combination with trastuzumab and chemotherapy 0
3,020 		100% Indication of minor additional benefit repealed
Bictegravir / Emtricitabin / Tenofoviralafenamid (1) Biktarvy® Gilead Sciecnes GmbH Infectious diseases HIV infection 66,800 100% additional benefit not proven
Velmanase alfa (1) Lamzede® Chiesi GmbH Metabolic diseases Alpha mannosidosis 70–140 100% non-quantifiable additional benefit Orphan
Olaparib (2) Lynparza® AstraZeneca GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, platinum-sensitive, maintenance therapy 1,900–2,400 100% Hint for minor additional benefit
Cabozantinib (Cabometyx, 3) Cabometyx® Ipsen Pharma GmbH Oncological diseases Renal cell carciRenal cell carcinoma (RCC), first-linenoma (RCC) 2,240–2,570 100% additional benefit not proven
Dolutegravir / Rilpivirin (1) Juluca® ViiV Healthcare GmbH Infectious diseases HIV infection 53,000 100% additional benefit not proven
Bosutinib (2) Bosulif® Pfizer Pharma GmbH Oncological diseases Chronic myeloid leukaemia (CML), Ph+, first-line 0
690–810 		100% additional benefit not proven repealed
Darvadstrocel (1) Alofisel® Takeda Pharma Vertrieb GmbH & Co. KG Digestive system diseases Anal fistulas in Crohn's disease 90–230 100% non-quantifiable additional benefit Orphan
Extrakt aus Cannabis Sativa (2) Sativex® Almirall Hermal GmbH Nervous system diseases Multiple sclerosis (MS), spasticity 13,400–38,500 100% Indication of minor additional benefit
Tofacitinib (2) Xeljanz® Pfizer Pharma GmbH Musculoskeletal system diseases Rheumatoid arthritis (RA) 0
29,490–63,815 		100% additional benefit not proven repealed
Rurioctocog alfa pegol (1) Adynovi® Shire Deutschland GmbH Hematopoietic diseases Hemophilia A, ≥ 12 years 2,840–3,190 100% additional benefit not proven
Hydrocortison (1) Alkindi® Diurnal Ltd. Metabolic diseases Adrenal insufficiency, < 18 years 780–1,180 100% additional benefit not proven
Ertugliflozin / Sitagliptin (1) Steglujan® MSD Sharp & Dohme GmbH Metabolic diseases Diabetes mellitus type 2 326,100–341,100 100% additional benefit not proven
Cariprazin (1) Reagila® Recordati Pharma GmbH Mental illnesses Schizophrenia 224,000–299,000 21% Indication of minor additional benefit
Burosumab (1) Crysvita® Kyowa Kirin GmbH Metabolic diseases X-linked hypophosphatemia (XLH), ≥ 1 to < 18 years 0
200–500 		100% non-quantifiable additional benefit Orphan repealed
Bezlotoxumab (1) Zinplava® MSD Sharp & Dohme GmbH Infectious diseases Prevention of recurrent Clostridium difficile infection 6,800–37,600 100% Indication of minor additional benefit
Emicizumab (1) Hemlibra® Roche Pharma AG Hematopoietic diseases Hemophilia A 100 50% Hint for non-quantifiable additional benefit
Patiromer (1) Veltassa® Fresenius Medical Care Nephrologica Deutschland GmbH Metabolic diseases Hyperkalemia 61,700 100% additional benefit not proven
Glycopyrroniumbromid (1) Sialanar® Proveca Limited Other diseases Sialorrhea 2,800–3,100 100% Hint for non-quantifiable additional benefit
Evolocumab (2) Repatha® Amgen GmbH Metabolic diseases Primary hypercholesterolaemia, mixed dyslipidaemia 271,500 100% additional benefit not proven
Fluticasonfuroat / Umeclidinium / Vilanterol (1) Trelegy Ellipta® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 404,000–1,227,000 100% additional benefit not proven
Glycerolphenylbutyrat (1) Ravicti® Swedish Orphan Biovitrum GmbH Metabolic diseases Urea cycle disorders (UCD), ≥ 2 months 100–250 100% non-quantifiable additional benefit Orphan
Ixekizumab (2) Taltz® Lilly Deutschland GmbH Skin diseases Psoriatic arthritis (PA) 29,100 35% Hint for minor additional benefit
Insulin glargin / Lixisenatid (1) Suliqua® Sanofi-Aventis Deutschland GmbH Metabolic diseases Diabetes mellitus type 2 461,400–472,500 100% additional benefit not proven
Sonidegib (1) Odomzo® Sun Pharmaceuticals Germany GmbH Oncological diseases Basal cell carcinoma (BCC) 130–350 100% additional benefit not proven
Benralizumab (1) Fasenra® AstraZeneca GmbH Respiratory system diseases Bronchial asthma 6,800–80,000 50% Hint for minor additional benefit
Letermovir (1) Prevymis® MSD SHARP & DOHME GMBH Infectious diseases Cytomegalovirus infection 0
1,000–1,800 		100% non-quantifiable additional benefit Orphan repealed
Fluticasonfuroat / Vilanterol-Trifenatat (2) Relvar Ellipta® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Bronchial asthma n.d. 100% additional benefit not proven
Ipilimumab (3) Yervoy® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, ≥ 12 to < 18 years 1–5 100% additional benefit not proven
Ocrelizumab (1) Ocrevus® Roche Pharma AG Nervous system diseases Multiple sclerosis (MS) 165,300–182,200 81% Proof of minor additional benefit
Lumacaftor / Ivacaftor (2) Orkambi® Vertex Pharmaceuticals (Europe) Limited Metabolic diseases Cystic fibrosis (CF), homozygous F508del mutation in CFTR gene, ≥ 6 years 500 100% Hint for non-quantifiable additional benefit
Brentuximab Vedotin (3) Adcetris® Takeda GmbH Oncological diseases Cutaneous T-cell lymphoma, CD30+ 40–130 100% minor additional benefit Orphan
Elvitegravir / Cobicistat / Emtricitabin / Tenofoviralafenamid (2) Genvoya® Gilead Sciences GmbH Infectious diseases HIV infection, 6 to < 12 years 100 100% additional benefit not proven
Allogene, genetisch modifizierte T-Zellen (1) Zalmoxis® Dompé farmaceutici S.p.A. Oncological diseases Haematological malignancies, concomitant therapy in haploidentical haematopoietic stem cell transplantation 100–140 100% non-quantifiable additional benefit Orphan
Alectinib (2) Alecensa® Roche Pharma AG Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, first-line 350–850 100% Hint for non-quantifiable additional benefit
Dapagliflozin / Metformin (2) Xigduo® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2 0
468,700 		100% additional benefit not proven repealed
Dapagliflozin (2) Forxiga® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2 0
468,700 		100% additional benefit not proven repealed
Abirateronacetat (3) Zytiga® Janssen-Cilag GmbH Oncological diseases Prostate carcinoma (PC), high-risk, combination with androgen deprivation therapy 1,500–2,200 100% Indication of considerable additional benefit
Niraparib (1) Zejula® TESARO Bio Germany GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma 0
1,900–2,400 		100% non-quantifiable additional benefit Orphan repealed
Cladribin (1) Mavenclad® Merck Serono GmbH Nervous system diseases Highly active relapsing multiple sclerosis (MS) 40,300–41,000 100% additional benefit not proven
Dupilumab (1) Dupixent® Sanofi-Aventis Deutschland GmbH Skin diseases Atopic dermatitis (AD) 52,000 100% Indication of considerable additional benefit
Guselkumab (1) Tremfya® Janssen-Cilag GmbH Skin diseases Plaque psoriasis (PP) 52,200–234,400 45% Proof of considerable additional benefit
Perampanel (3) Fycompa® Eisai GmbH Nervous system diseases Epilepsy, primary generalised seizures, ≥ 12 years 15,600–50,700 100% additional benefit not proven
Cenegermin (1) Oxervate® Dompé farmaceutici S.p.A Eye diseases Keratitis 1,730–3,170 100% non-quantifiable additional benefit Orphan
Elvitegravir / Cobicistat / Emtricitabin / Tenofovirdisoproxil (2) Stribild® Gilead Sciences GmbH Infectious diseases HIV infection, 12 to < 18 years 100 100% additional benefit not proven
Tivozanib (1) Fotivda® EUSA Pharma GmbH Oncological diseases Renal cell carcinoma (RCC) 2,953–7,126 100% additional benefit not proven
Nonacog beta pegol (1) Refixia® Novo Nordisk Pharma GmbH Hematopoietic diseases Hemophilia B, ≥ 12 years 500–570 100% additional benefit not proven
Cabozantinib (Cabometyx, 2) Cabometyx® Ipsen Pharma GmbH Oncological diseases Renal cell carcinoma (RCC), after VEGF pre-therapy 1,200–3,300 100% Indication of minor additional benefit
Telotristatethyl (1) Xermelo® Ipsen Pharma GmbH Oncological diseases Carcinoid tumor, neuroendocrine tumors, combination with SAA therapy 300–1,000 100% non-quantifiable additional benefit Orphan
Midostaurin (1) Rydapt® Novartis Pharma GmbH Oncological diseases Acute myeloid leukaemia (AML); Systemic mastocytosis (ASM) 0
400–710 		80% considerable additional benefit Orphan repealed
Obinutuzumab (3) Gazyvaro® Roche Pharma AG Oncological diseases Follicular lymphoma (FL), first-line 0
1,300–1,500 		100% non-quantifiable additional benefit Orphan repealed
Sofosbuvir (2) Sovaldi® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C, 12 to < 18 years 1,025 100% Hint for non-quantifiable additional benefit
Ribociclib (1) Kisqali® Novartis Pharma GmbH Oncological diseases Breast cancer (BC) HR+, HER2-, postmenopausal women, combination with aromatase inhibitor 0
7,180–34,790 		100% additional benefit not proven repealed
Pembrolizumab (5) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Urothelial carcinoma (UC) 0
2,300–3,300 		61% Indication of considerable additional benefit repealed
Elosulfase alfa (2) Vimizim® BioMarin Deutschland GmbH Metabolic diseases Mucopolysaccharidosis (type IVA) 20–100 100% minor additional benefit Orphan
Atezolizumab (1) Tecentriq® Roche Pharma AG Oncological diseases Non-small cell lung carcinoma (NSCLC), after prior chemotherapy 18,800–34,600 40% Indication of considerable additional benefit
Atezolizumab (2) Tecentriq® Roche Pharma AG Oncological diseases Urothelial carcinoma (UC), first-line 1,500–1,900
2,300–3,300 		61% Hint for minor additional benefit repealed subpopulations
Avelumab (1) Bavencio® Merck Serono GmbH/Pfizer Pharma GmbH Oncological diseases Merkel-cell carcinoma (MCC) 0
160–410 		100% non-quantifiable additional benefit Orphan repealed
Dimethylfumarat (Skilarence, 1) Skilarence® Almirall Hermal GmbH Skin diseases Plaque psoriasis (PP) 52,200–234,100 100% additional benefit not proven
Darunavir / Cobicistat / Emtricitabin / Tenofoviralafenamid (1) Symtuza® Janssen-Cilag GmbH Infectious diseases HIV infection 62,050 100% additional benefit not proven
Brodalumab (1) Kyntheum® Leo Pharma GmbH Skin diseases Plaque psoriasis (PP) 52,200–234,400 45% Indication of non-quantifiable additional benefit
Carfilzomib (3) Kyprolis® Amgen GmbH Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with dexamethasone or lenalidomide and dexamethasone 4,700–7,000 100% Hint for considerable additional benefit Orphan (turnover limit)
Sarilumab (1) Kevzara® Sanofi-Aventis Deutschland GmbH Musculoskeletal system diseases Rheumatoid arthritis (RA) 87,300–184,470 34% Hint for considerable additional benefit
Sofosbuvir / Velpatasvir / Voxilaprevir (1) Vosevi® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C 104,600 100% additional benefit not proven
Daratumumab (3) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma (MM), monotherapy; multiple myeloma, at least 1 prior therapy, combination with lenalidomide and dexamethasone or bortezomib and dexamethasone 2,300
7,000–9,300 		72% Indication of considerable additional benefit Orphan (turnover limit) repealed subpopulations
Ledipasvir / Sofosbuvir (2) Harvoni® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C, 12 to < 18 years 5,300 50% Hint for non-quantifiable additional benefit
Saxagliptin / Metformin (4) Komboglyze® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2, combination therapy other than insulin and sulphonylurea 77,400 100% additional benefit not proven
Glecaprevir / Pibrentasvir (1) Maviret® AbbVie Deutschland GmbH & Co. KG Infectious diseases Chronic hepatitis C 104,600 100% additional benefit not proven
Ceritinib (3) Zykadia® Novartis Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, first-line 430–850 100% additional benefit not proven
Inotuzumab ozogamicin (1) Besponsa® Pfizer Pharma GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL) 120–200 100% minor additional benefit Orphan
Nivolumab (9) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Urothelial carcinoma (UC) 1,500–1,900 100% additional benefit not proven
Nusinersen (1) Spinraza® Biogen GmbH Nervous system diseases Spinal muscular atrophy (SMA) 0
840–1,060 		10% major additional benefit Orphan repealed
Cerliponase alfa (1) Brineura® BioMarin Deutschland GmbH Metabolic diseases Neuronal ceroid lipofuscinosis type 2 0
20–40 		100% non-quantifiable additional benefit Orphan repealed
Blinatumomab (2) Blincyto® Amgen GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL) 60–170 100% considerable additional benefit Orphan
Nivolumab (8) Opdivo® Bristol-Myers-Squibb GmbH & Co. KGaA Oncological diseases Melanoma, BRAF V600 wild-type, first-line, combination with ipilimumab 0
500–1,500 		100% additional benefit not proven repealed
Etelcalcetid (1) Parsabiv® Amgen GmbH Metabolic diseases Secondary hyperparathyroidism (HPT) 21,896–26,360 100% additional benefit not proven
Nivolumab (7) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Squamous cell carcinoma head and neck 970–6,850 77% Hint for considerable additional benefit
Rolapitant (1) Varuby® TESARO Bio Germany GmbH Other diseases Nausea and vomiting due to chemotherapy 157,700–279,700 100% additional benefit not proven
Pembrolizumab (4) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Hodgkin lymphoma (HL) 60–180 100% additional benefit not proven
Lutetium-(177Lu)-Oxodotreotid (1) Lutathera® Advanced Accelerator Applications Germany GmbH Oncological diseases Radiotherapy n.d. exempted Orphan
Landiololhydrochlorid (1) Rapibloc® Amomed Pharma GmbH Cardiovascular diseases Tachycardia, atrial fibrillation or atrial flutter n.d. exempted
Dinutuximab beta (1) Dinutuximab beta EUSA® EUSA Pharma (Germany) GmbH Oncological diseases Other antineoplastic agents n.d. exempted Orphan
Dabrafenib (3) Tafinlar® Novartis Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), BRAF V600 mutation, combination with trametinib 230–820 100% additional benefit not proven
Trametinib (2) Mekinist® Novartis Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), BRAF V600 mutation 230–720 100% additional benefit not proven
Tofacitinib (1) Xeljanz® Pfizer Pharma GmbH Musculoskeletal system diseases Rheumatoid arthritis (RA) 0
87,300–184,470 		100% additional benefit not proven repealed
Alectinib (1) Alecensa® Roche Pharma AG Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, pre-treated with crizotinib 200–1,310 81% Hint for minor additional benefit
Osimertinib (2) Tagrisso® AstraZeneca GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), T790M EGFR mutation 560–2,730 100% Hint for considerable additional benefit
Axitinib (2) Inlyta® Pfizer Pharma GmbH Oncological diseases Renal cell carcinoma (RCC), after failure of sunitinib or a cytokine theryapy 483–2,406 0.3% Hint for minor additional benefit
Tenofoviralafenamid (1) Vemlidy® Gilead Sciences GmbH Infectious diseases Chronic hepatitis B, ≥ 12 years 0
12,400–38,100 		100% additional benefit not proven repealed
Dolutegravir (2) Tivicay® ViiV Healthcare GmbH Infectious diseases HIV infection, 6 to < 12 years 110 100% additional benefit not proven
Baricitinib (1) Olumiant® Lilly Deutschland GmbH Musculoskeletal system diseases Rheumatoid arthritis (RA) 87,300–184,470 100% additional benefit not proven
Secukinumab (3) Cosentyx® Novartis Pharma GmbH Skin diseases Plaque psoriasis (PP) 19,800–137,300 100% Indication of considerable additional benefit
Ixekizumab (1) Taltz® Lilly Deutschland GmbH Skin diseases Plaque psoriasis (PP) 52,200–234,400 55% Indication of considerable additional benefit
Pembrolizumab (3) Keytruda® MSD SHARP & DOHME GMBH Oncological diseases Non-small cell lung carcinoma (NSCLC), first-line 4,000 100% Indication of considerable additional benefit
Lonoctocog alfa (1) Afstyla® CSL Behring GmbH Hematopoietic diseases Hemophilia A 3,190–3,590 100% additional benefit not proven
Obeticholsäure (1) Ocaliva® Intercept Pharma Deutschland GmbH Digestive system diseases Biliary cirrhosis 0
1,050–7,350 		100% non-quantifiable additional benefit Orphan repealed
Reslizumab (1) Cinqaero® Teva GmbH Respiratory system diseases Bronchial asthma 4,800–12,000 50% Hint for minor additional benefit
Vandetanib (3) Caprelsa® Genzyme GmbH Oncological diseases Thyroid carcinoma, ≥ 5 years 0
2–8 		100% Hint for non-quantifiable additional benefit repealed
Ixazomib (1) Ninlaro® Takeda GmbH Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with lenalidomide and dexamethasone 0
4,700–7,000 		100% non-quantifiable additional benefit Orphan repealed
Daratumumab (2) Darzalex® Janssen Cilag GmbH Oncological diseases Multiple myeloma (MM) n.d. discontinued Orphan
Elbasvir / Grazoprevir (1) Zepatier® MSD Sharp & Dohme GmbH Infectious diseases Chronic hepatitis C 64,800 100% additional benefit not proven
Nivolumab (6) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Hodgkin lymphoma (HL) 40–90 100% additional benefit not proven
Venetoclax (1) Venclyxto® AbbVie Deutschland GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), monotherapy 0
300–700 		100% non-quantifiable additional benefit Orphan repealed
Palbociclib (1) Ibrance® Pfizer Pharma GmbH Oncological diseases Breast cancer (BC) 7,380–35,760
14,560–70,550 		100% additional benefit not proven repealed subpopulations
Olaratumab (1) Lartruvo® Lilly Deutschland GmbH Oncological diseases Soft tissue sarcoma 0
1,200–1,400 		100% considerable additional benefit Orphan repealed
Cabozantinib (Cabometyx, 1) Cabometyx® Ipsen Pharma GmbH Oncological diseases Renal cell carcinoma (RCC) 0
1,200–3,300 		100% Hint for non-quantifiable additional benefit repealed
Macitentan (2) Opsumit® Actelion Pharmaceuticals Deutschland GmbH Cardiovascular diseases Pulmonary arterial hypertension (PAH) 580–7,850 100% additional benefit not proven Orphan (turnover limit)
Opicapon (1) Ongentys® Bial-Portela & Ca, S.A. Nervous system diseases Morbus Parkinson 45,200–61,100 100% additional benefit not proven
Ceritinib (2) Zykadia® Novartis Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, pre-treated with crizotinib 200–1,310 81% Hint for considerable additional benefit
Crizotinib (4) Xalkori® Pfizer Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ROS1+ 210–770 100% additional benefit not proven
Lenvatinib (Kisplyx, 1) Kisplyx® Eisai GmbH Oncological diseases Renal cell carcinoma (RCC) 0
1,200–3,300 		100% Hint for minor additional benefit repealed
Idelalisib (3) Zydelig® Gilead Sciences GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), combination with ofatumumab; Chronic lymphocytic leukaemia (CLL), first-line, 17p deletion/TP53 mutation, combination with rituximab 2,020–7,560 0.4% Hint for non-quantifiable additional benefit
Ibrutinib (5) Imbruvica® Janssen-Cilag GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), at least 1 prior therapy, combination with bendamustine and rituximab 1,500–5,600 50% Hint for considerable additional benefit Orphan (turnover limit)
Pembrolizumab (2) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), after prior chemotherapy 12,500–24,300 40% Indication of considerable additional benefit
Trifluridin / Tipiracil (1) Lonsurf® Servier Deutschland GmbH Oncological diseases Colorectal carcinoma (CRC), pre-treated patients 0
6,900–12,200 		100% Hint for minor additional benefit repealed
Teduglutid (2) Revestive® Shire Deutschland GmbH Digestive system diseases Short bowel syndrome, 1 to < 18 years 230–500 100% non-quantifiable additional benefit Orphan
Tasimelteon (1) Hetlioz® Vanda Pharmaceuticals Inc. Nervous system diseases Sleep disorders (sleep-wake rhythm), blind adults 7,000 100% non-quantifiable additional benefit Orphan
Pitolisant (1) Wakix® Bioprojet Pharma SARL Nervous system diseases Narcolepsy 15,000–30,000 100% non-quantifiable additional benefit Orphan
Brentuximab Vedotin (2) Adcetris® Takeda GmbH Oncological diseases Hodgkin lymphoma (HL), CD30+, increased risk of recurrence or progression after transplantation 40–60 100% non-quantifiable additional benefit Orphan
Carfilzomib (2) Kyprolis® Amgen GmbH Oncological diseases Multiple myeloma (MM), combination with dexamethasone 0
4,700–7,000 		100% minor additional benefit Orphan repealed
Emtricitabin / Rilpivirin / Tenofoviralafenamid (1) Odefsey® Gilead Sciences GmbH Infectious diseases HIV infection 56,800–59,300 100% additional benefit not proven
Sofosbuvir / Velpatasvir (1) Epclusa® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C 100,000 28% Hint for considerable additional benefit
Ibrutinib (4) Imbruvica® Janssen-Cilag GmbH Oncological diseases Chronic lymphocytic leukemia (CLL), first-line 2,840 100% additional benefit not proven Orphan (turnover limit)
Sitagliptin / Metformin (2) Janumet, Velmetia® MSD SHARP & DOHME GMBH Metabolic diseases Diabetes mellitus type 2, monotherapy or combination with sulphonylurea or insulin 792,050–810,850 100% additional benefit not proven
Sitagliptin (2) Januvia, Xelevia® MSD SHARP & DOHME GMBH Metabolic diseases Diabetes mellitus type 2, monotherapy or combination with metformin, sulphonylurea or PPARɣ 1,070,800–1,270,800
1,705,400–1,905,400 		35% Hint for minor additional benefit repealed subpopulations
Saxagliptin / Metformin (3) Komboglyze® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2, monotherapy or combination with insulin 729,650–748,450 100% additional benefit not proven
Saxagliptin (3) Onglyza® AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2, combination therapy 1,182,900–1,382,900 100% additional benefit not proven
Crizotinib (3) Xalkori® Pfizer Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, pre-treated 480 71% Hint for considerable additional benefit
Obinutuzumab (2) Gazyvaro® Roche Pharma AG Oncological diseases Follicular lymphoma (FL) 0
790–940 		100% non-quantifiable additional benefit Orphan repealed
Nivolumab (5) Opdivo® Bristol-Myers-Squibb GmbH & Co. KGaA Oncological diseases Melanoma, combination with ipilimumab 2,230–3,690
2,500–4,500 		100% additional benefit not proven repealed subpopulations
Selexipag (1) Uptravi® Actelion Pharmaceuticals Deutschland GmbH Cardiovascular diseases Pulmonary arterial hypertension (PAH) 580–7,850 100% additional benefit not proven
Eftrenonacog alfa (1) Alprolix® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Hemophilia B 0
580–660 		100% non-quantifiable additional benefit Orphan repealed
Talimogen laherparepvec (1) Imlygic® Amgen GmbH Oncological diseases Melanoma, stage IIIB, IIIC, IVMI1a 375–620 100% additional benefit not proven
Eribulin (3) Halaven® Eisai GmbH Oncological diseases Liposarcoma 30–150 50% Hint for considerable additional benefit
Albutrepenonacog alfa (1) Idelvion® CSL Behring GmbH Hematopoietic diseases Hemophilia B 0
580–660 		100% non-quantifiable additional benefit Orphan repealed
Migalastat (1) Galafold® Amicus Therapeutics GmbH Metabolic diseases Fabry disease 0
20–490 		100% non-quantifiable additional benefit Orphan repealed
Elotuzumab (1) Empliciti® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with lenalidomide and dexamethasone 4,700–7,000 100% Hint for minor additional benefit
Ataluren (2) Translarna® PTC Therapeutics International Limited Musculoskeletal system diseases Duchenne muscular dystrophy (DMD), ≥ 5 years 0
30–40 		100% minor additional benefit Orphan repealed
Daratumumab (1) Darzalex® Janssen-Cilag GmbH Oncological diseases Multiple myeloma (MM), monotherapy, pre-treated patients 0
2,300 		100% non-quantifiable additional benefit Orphan repealed
Emtricitabin / Tenofoviralafenamid (1) Descovy® Gilead Sciences GmbH Infectious diseases HIV infection 63,000 100% additional benefit not proven
Ospemifen (1) Senshio® Shionogi GmbH Genitourinary system diseases Vulvovaginal atrophy 133,400–151,800 100% additional benefit not proven
Ramucirumab (4) Cyramza® Lilly Deutschland GmbH Oncological diseases Gastric or gastro-oesophageal junction adenocarcinoma; combination with paclitaxel 5,900–7,900 50% Hint for minor additional benefit
Afatinib (3) Giotrif® Boehringer Ingelheim Pharma GmbH & Co. KG Oncological diseases Non-small cell lung carcinoma (NSCLC), squamous cell histology 6,720–12,420 100% additional benefit not proven
Nivolumab (3) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Renal cell carcinoma (RCC) 1,200–3,300 92% Indication of considerable additional benefit
Nivolumab (4) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Non-small cell lung carcinoma (NSCLC), non-squamous cell histology, after previous chemotherapy 11,830–21,830 40% Indication of considerable additional benefit
Osimertinib (1) Tagrisso® AstraZeneca GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), T790M EGFR mutation 245–1,465
475–2,830 		100% additional benefit not proven repealed subpopulations
Necitumumab (1) Portrazza® Lilly Deutschland GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC) 6,300–7,700 100% additional benefit not proven
Ticagrelor (2) Brilique® AstraZeneca GmbH Hematopoietic diseases Prevention of atherothrombotic events in patients with myocardial infarction and high risk for an atherothrombotic event 105,000 100% Hint for minor additional benefit
Idelalisib (2) Zydelig® Gilead Sciences GmbH Oncological diseases Chronic lymphocytic leukemia (CLL) 2,000–7,500
2,200–7,800 		24% Hint for non-quantifiable additional benefit repealed subpopulations
Empagliflozin / Metformin (1) Synjardy® Boehringer Ingelheim Pharma GmbH & Co. KG Metabolic diseases Diabetes mellitus type 2 615,299–615,301 100% additional benefit not proven
Empagliflozin (2) Jardiance® Boehringer Ingelheim Pharma GmbH & Co. KG Metabolic diseases Diabetes mellitus type 2 1,253,400–1,453,400 42% Hint for considerable additional benefit
Ramucirumab (2) Cyramza® Lilly Deutschland GmbH Oncological diseases Colorectal carcinoma (CRC) 2,900–7,300 100% additional benefit not proven
Ramucirumab (3) Cyramza® Lilly Deutschland GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC) 5,900–15,400 100% additional benefit not proven
Afamelanotid (1) Scenesse® Clinuvel (UK) Limited Metabolic diseases Erythropoietic protoporphyria 0
540–1,090 		100% non-quantifiable additional benefit Orphan repealed
Brivaracetam (1) Briviact® UCB Pharma GmbH Nervous system diseases Epilepsy, focal seizures, ≥ 16 years 86,000–200,000 100% additional benefit not proven
Vismodegib (2) Erivedge® Roche Pharma AG Oncological diseases Basal cell carcinoma (BCC) 295 95% Hint for minor additional benefit
Umeclidinium (1) Incruse® GlaxoSmithKline GmbH & Co KG Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 2,342,000–2,765,000 100% additional benefit not proven
Ibrutinib (3) Imbruvica® Janssen-Cilag GmbH Oncological diseases Mantle cell lymphoma (MCL), chronic lymphocytic leukaemia (CLL), Waldenström's disease 3,780–11,100 12% Indication of considerable additional benefit Orphan (turnover limit)
Mepolizumab (1) Nucala® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Bronchial asthma 16,000–100,000 50% Hint for minor additional benefit
Rilpivirin (2) Edurant® Janssen-Cilag GmbH Infectious diseases HIV infection, 12 to < 18 years 10 100% additional benefit not proven
Sacubitril / Valsartan (1) Entresto® Novartis Pharma GmbH Cardiovascular diseases Symptomatic chronic heart failure 550,000–1,350,000 68% Hint for considerable additional benefit
Efmoroctocog alfa (1) Elocta® Swedish Orphan Biovitrum GmbH Hematopoietic diseases Hemophilia A 3,190–3,590 100% additional benefit not proven
Elvitegravir / Cobicistat / Emtricitabin / Tenofoviralafenamid (1) Genvoya® Gilead Sciences GmbH Infectious diseases HIV infection 56,800 100% additional benefit not proven
Crizotinib (2) Xalkori® Pfizer Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, first-line 0
300–900 		100% Hint for considerable additional benefit repealed
Secukinumab (2) Cosentyx® Novartis Pharma GmbH Musculoskeletal system diseases Psoriatic arthritis (PA), Radiographic axial spondyloarthritis (AS)Psoriatic arthritis (PA), Bekhterev's disease 40,400–141,600 100% additional benefit not proven
Blinatumomab (1) Blincyto® Amgen GmbH Oncological diseases B-cell acute lymphoblastic leukaemia (ALL) 0
60–170 		100% non-quantifiable additional benefit Orphan repealed
Carfilzomib (1) Kyprolis® Amgen GmbH Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with dexamethasone or lenalidomide and dexamethasone 0
4,700–7,000 		100% non-quantifiable additional benefit Orphan repealed
Cobimetinib (1) Cotellic® Roche Pharma AG Oncological diseases Melanoma, BRAF V600 mutation, combination with vemurafenib 1,400 100% Indication of considerable additional benefit
Ivacaftor (3) Kalydeco® Vertex Pharmaceuticals (Europe) Limited Metabolic diseases Cystic fibrosis (CF), R117H mutation, 2 to 5 years, > 18 years 0
59 		75% minor additional benefit Orphan repealed
Lumacaftor / Ivacaftor (1) Orkambi® Vertex Pharmaceuticals (Europe) Limited Metabolic diseases Cystic fibrosis (CF), > 12 years, homozygous F508del mutation 2,600 100% Indication of considerable additional benefit
Aflibercept (5) Eylea® Bayer Vital GmbH Eye diseases Myopic choroidal neovascularisation 27,500–80,000 100% additional benefit not proven
Fingolimod (4) Gilenya® Novartis Pharma GmbH Nervous system diseases Highly active relapsing-remitting multiple sclerosis (MS), after inadequate pre-treatment 14,000–16,000 100% additional benefit not proven
Alirocumab (1) Praluent® Sanofi-Aventis Deutschland GmbH Metabolic diseases Hypercholesterolemia or mixed dyslipidaemia 1,500
273,250 		100% additional benefit not proven repealed subpopulations
Isavuconazol (1) Cresemba® Basilea Pharmaceutica International Ltd. Infectious diseases Aspergillosis, mucormycosis 1,293–5,342 100% non-quantifiable additional benefit Orphan
Aclidiniumbromid (2) Eklira Genuair, Bretaris Genuair® AstraZeneca GmbH Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 2,342,800–2,768,200 6% Indication of considerable additional benefit
Trametinib (1) Mekinist® Novartis Pharma GmbH Oncological diseases Melanoma, BRAF V600 mutation 1,400 50% Indication of considerable additional benefit
Sebelipase alfa (1) Kanuma® Synageva BioPharma Limited Metabolic diseases Lysosomal acid lipase deficiency 0
31–843 		100% non-quantifiable additional benefit Orphan repealed
Regorafenib (3) Stivarga® Bayer Vital GmbH Oncological diseases Colorectal carcinoma (CRC) 6,900–12,200 100% additional benefit not proven
Pomalidomid (2) Imnovid® Celgene GmbH Oncological diseases Multiple myeloma (MM), at least 2 prior therapies, combination with dexamethasone 2,300 50% Hint for considerable additional benefit Orphan (turnover limit)
Panobinostat (1) Farydak® Novartis Pharma GmbH Oncological diseases Multiple myeloma (MM), at least 1 prior therapy, combination with pomalidomide and dexamethasone 2,300 100% non-quantifiable additional benefit Orphan
Idebenon (1) Raxone® Santhera Pharmaceuticals Eye diseases Leber hereditary optic neuropathy 0
1,500–3,000 		100% non-quantifiable additional benefit Orphan repealed
Dabrafenib (2) Tafinlar® Novartis Pharma GmbH Oncological diseases Melanoma, BRAF V600 mutation, combination with trametinib 1,400 100% Indication of considerable additional benefit
Asfotase alfa (1) Strensiq® Alexion Europe SAS Metabolic diseases Hypophosphatasia (HPP) 0
1,000 		100% non-quantifiable additional benefit Orphan repealed
Evolocumab (1) Repatha® Amgen GmbH Metabolic diseases Primary hypercholesterolaemia, mixed dyslipidaemia 1,810–1,819
273,310–273,319 		100% additional benefit not proven repealed subpopulations
Pertuzumab (2) Perjeta® Roche Pharma AG Oncological diseases Breast cancer (BC), inflammatory or early with high risk of recurrence, neoadjuvant, combination with trastuzumab and chemotherapy 2,900–4,850 100% additional benefit not proven
Insulin degludec / Liraglutid (2) Xultophy® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 2, combination with oral antidiabetics 78,500–170,000 100% additional benefit not proven
Tiotropium / Olodaterol (1) Spiolto Respimat® Boehringer Ingelheim Pharma GmbH & Co. KG Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 2,342,000–2,780,000 92% Indication of minor additional benefit
Pembrolizumab (1) Keytruda® MSD Sharp & Dohme GmbH Oncological diseases Melanoma 2,500–4,500 71% Indication of considerable additional benefit
Netupitant / Palonosetron (1) Akynzeo® RIEMSER Pharma GmbH Other diseases Nausea and vomiting due to chemotherapy 157,700–279,700 100% additional benefit not proven
Nivolumab (2) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Non-small cell lung carcinoma (NSCLC), squamous cell histology, after previous chemotherapy 4,200–6,000 87% Indication of considerable additional benefit
Gaxilose (1) LacTest® VENTER PHARMA S.L. Metabolic diseases Diagnosis of hypolactasia 180,000–230,000 100% additional benefit not proven
Edoxaban (1) Lixiana® Daiichi Sankyo Deutschland GmbH Hematopoietic diseases Prophylaxis of thromboembolic events 1,167,000–1,334,000 81% Indication of minor additional benefit
Nivolumab (1) Opdivo® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma 2,500–4,500 15% Indication of considerable additional benefit
Belatacept (2) Nulojix® Bristol-Myers Squibb GmbH & Co. KGaA Genitourinary system diseases Prophylaxis of graft rejection (GvHD) after kidney transplantation 2,950–3,380 100% Indication of considerable additional benefit
Lenvatinib (1) Lenvima® Eisai GmbH Oncological diseases Thyroid carcinoma (DTC) 0
600–815 		100% non-quantifiable additional benefit Orphan repealed
Ceritinib (1) Zykadia® Novartis Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, pre-treated with crizotinib 0
120–560 		100% additional benefit not proven repealed
Lomitapid (2) Lojuxta® Aegerion Pharmaceuticals GmbH Metabolic diseases Hypercholesterolemia 60–69 100% additional benefit not proven
Secukinumab (1) Cosentyx® Novartis Pharma GmbH Skin diseases Plaque psoriasis (PP) 32,400–97,100
52,200–234,400 		8% Indication of considerable additional benefit repealed subpopulations
Olaparib (1) Lynparza® AstraZeneca GmbH Oncological diseases Ovarian carcinoma, fallopian tube carcinoma or primary peritoneal carcinoma, platinum-sensitive, maintenance therapy 0
200–600 		100% non-quantifiable additional benefit Orphan repealed
Ivermectin (1) Soolantra® Galderma Laboratorium GmbH Skin diseases Inflammatory lesions of rosacea 0
309,000–703,000 		100% additional benefit not proven repealed
Afatinib (2) Giotrif® Boehringer Ingelheim Pharma GmbH & Co. KG Oncological diseases Non-small cell lung carcinoma (NSCLC), EGFR mutation, EGFR-TKI-naive patients 3,670–8,590 42% Indication of major additional benefit
Safinamid (1) Xadago® Zambon S.p.A. Nervous system diseases Morbus Parkinson 45,200–61,100 100% additional benefit not proven
Ibrutinib (2) Imbruvica® Janssen-Cilag GmbH Oncological diseases Waldenström's disease n.d. discontinued Orphan
Vortioxetin (1) Brintellix® Lundbeck GmbH Mental illnesses Major depression 3,369,000–3,669,000 100% additional benefit not proven
Insulin degludec / Liraglutid (1) Xultophy® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 2 752,000–903,000 100% additional benefit not proven
Ruxolitinib (3) Jakavi® Novartis Pharma GmbH Oncological diseases Polycythaemia vera 240–1,470 100% Hint for considerable additional benefit Orphan (turnover limit)
Fingolimod (3) Gilenya® Novartis Pharma GmbH Nervous system diseases Rapidly progressing relapsing-remitting multiple sclerosis (MS) 4,600–12,300
17,100–24,800 		21% Indication of considerable additional benefit repealed subpopulations
Eliglustat (1) Cerdelga® Genzyme GmbH Metabolic diseases Sphingolipidoses (Gaucher disease type 1) 150–500 100% non-quantifiable additional benefit Orphan
Aflibercept (4) Eylea® Bayer Vital GmbH Eye diseases Macular edema following retinal branch vein occlusion 23,100–60,000 100% additional benefit not proven
Nintedanib (Ofev, 1) Ofev® Boehringer Ingelheim Pharma GmbH & Co KG Respiratory system diseases Idiopathic pulmonary fibrosis 0
2,080–19,700 		100% minor additional benefit Orphan repealed
Insulin degludec (3) Tresiba® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 1 and type 2, ≥ 1 to < 18 years 20,200 100% additional benefit not proven
Apremilast (1) Otezla® Celgene GmbH Skin diseases Plaque psoriasis (PP); Psoriatic arthritis (PA) 50,800–140,500 100% additional benefit not proven
Ombitasvir / Paritaprevir / Ritonavir (1) Viekirax® AbbVie Deutschland GmbH & Co. KG Infectious diseases Chronic hepatitis C 54,800 9% Indication of considerable additional benefit
Ramucirumab (1) Cyramza® Lilly Deutschland GmbH Oncological diseases Gastric or gastro-oesophageal junction adenocarcinoma; combination with paclitaxel 0
5,900–7,900 		100% minor additional benefit Orphan repealed
Dasabuvir (1) Exviera® AbbVie Deutschland GmbH & Co. KG Infectious diseases Chronic hepatitis C 61,700 24% Indication of considerable additional benefit
Dulaglutid (1) Trulicity® Lilly Deutschland GmbH Metabolic diseases Diabetes mellitus type 2 0
1,705,500–1,905,500 		31% Hint for minor additional benefit repealed
Aclidiniumbromid / Formoterol (1) Duaklir Genuair, Brimica Genuair® AstraZeneca GmbH Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 2,339,200–2,762,500 6% Indication of considerable additional benefit
Pasireotid (2) Signifor® Novartis Pharma GmbH Metabolic diseases Acromegaly 265–1,133 100% minor additional benefit Orphan
Nintedanib (1) Vargatef® Boehringer Ingelheim Pharma GmbH & Co. KG Oncological diseases Non-small cell lung carcinoma (NSCLC) 3,700–15,100 100% Indication of minor additional benefit
Enzalutamid (2) Xtandi® Astellas Pharma GmbH Oncological diseases Prostate carcinoma (PC), after androgen deprivation therapy, no indication for chemotherapy 15,000–28,800 100% Indication of considerable additional benefit
Tafluprost / Timolol (1) Taptiqom® Santen GmbH Eye diseases Reduction of intraocular pressure in patients with glaucoma/hypertension 95,600 100% additional benefit not proven
Ataluren (1) Translarna® PTC Therapeutics International Limited Musculoskeletal system diseases Duchenne muscular dystrophy (DMD), ≥ 5 years 0
82–110 		100% minor additional benefit Orphan repealed
Vildagliptin (2) Galvus, Jalra, Xiliarx® Novartis Pharma GmbH Metabolic diseases Diabetes mellitus type 2 35,900 100% additional benefit not proven
Ledipasvir / Sofosbuvir (1) Harvoni® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C 74,100 79% Hint for considerable additional benefit
Alipogentiparvovec (1) Glybera® Chiesi GmbH Metabolic diseases Familial lipoprotein lipase deficiency 17–35 100% non-quantifiable additional benefit Orphan
Simoctocog alfa (1) Nuwiq® Octapharma GmbH Hematopoietic diseases Hemophilia A 3,190–3,585 100% additional benefit not proven
Lurasidon (1) Latuda® Takeda GmbH Mental illnesses Schizophrenia 250,000 100% additional benefit not proven
Ibrutinib (1) Imbruvica® Janssen-Cilag GmbH Oncological diseases Mantle cell lymphoma (MCL), Chronic lymphocytic leukemia (CLL) 0
2,900–8,750 		100% non-quantifiable additional benefit Orphan repealed
Albiglutid (1) Eperzan® GlaxoSmithKline GmbH & Co. KG Metabolic diseases Diabetes mellitus type 2 1,705,400–1,905,400 35% Indication of minor additional benefit
Dolutegravir / Abacavir / Lamivudin (1) Triumeq® ViiV Healthcare GmbH Infectious diseases HIV infection, ≥ 12 years 54,398 9% Indication of considerable additional benefit
Sucroferric Oxyhydroxide (1) Velphoro® Fresenius Medical Care Deutschland GmbH Other diseases Serum phosphate level control in chronic kidney disease 56,600–71,500 100% additional benefit not proven
Sipuleucel-T (1) Provenge® Dendreon UK Limited Oncological diseases Prostate carcinoma (PC) 11,690–24,480 100% Hint for non-quantifiable additional benefit
Idelalisib (1) Zydelig® Gilead Sciences GmbH Oncological diseases Chronic lymphocytic leukaemia (CLL), Follicular lymphoma (FL) 1,800–7,050
3,000–11,100 		20% Hint for non-quantifiable additional benefit repealed subpopulations
Aflibercept (3) Eylea® Bayer Vital GmbH Eye diseases Diabetic macular edema 128,350–132,360 100% additional benefit not proven
Teduglutid (1) Revestive® NPS Pharma Germany GmbH Digestive system diseases Short bowel syndrome 1,100–2,400 100% minor additional benefit Orphan
Daclatasvir (1) Daklinza® Bristol-Myers Squibb GmbH & Co. KGaA Infectious diseases Chronic hepatitis C 70,500 1.4% Hint for considerable additional benefit
Regorafenib (2) Stivarga® Bayer Vital GmbH Oncological diseases Gastrointestinal stromal tumor (GIST) 100–700 100% additional benefit not proven
Propranolol (1) Hemangiol® Pierre Fabre Dermatologie Other diseases Infantile hemangioma 1,668–6,999 33% Indication of major additional benefit
Nalmefen (1) Selincro® Lundbeck GmbH Mental illnesses Reduction of alcohol consumption in alcohol dependence 220,000–248,000 100% additional benefit not proven
Ivacaftor (2) Kalydeco® Vertex Pharmaceuticals Limited Metabolic diseases Cystic fibrosis (CF), various mutations, ≥ 6 years 0
10–11 		100% minor additional benefit Orphan repealed
Apixaban (3) Eliquis® Bristol-Myers Squibb GmbH & Co. KGaA / Pfizer Deutschland GmbH Hematopoietic diseases Treatment and prophylaxis of venous thrombosis and pulmonary embolism 241,000 47% Indication of minor additional benefit
Obinutuzumab (1) Gazyvaro® Roche Pharma AG Oncological diseases Chronic lymphocytic leukemia (CLL) 0
818–1,477 		100% non-quantifiable additional benefit Orphan repealed
Empagliflozin (1) Jardiance® Boehringer Ingelheim Pharma GmbH & Co. KG Metabolic diseases Diabetes mellitus type 2 0
1,253,400–1,453,400 		100% additional benefit not proven repealed
Canagliflozin / Metformin (1) Vokanamet® Janssen-Cilag GmbH Metabolic diseases Diabetes mellitus type 2 615,300 100% additional benefit not proven
Cabozantinib (1) Cometriq® Swedish Orphan Biovitrum GmbH Oncological diseases Thyroid carcinoma (MTC) 0
60–500 		100% minor additional benefit Orphan repealed
Eribulin (2) Halaven® Eisai GmbH Oncological diseases Breast cancer (BC), after at least 2 chemotherapies; mammary carcinoma, after at least 1 chemotherapy 5,101–7,601 75% Hint for considerable additional benefit
Umeclidinium / Vilanterol (1) Anoro, Laventair® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 2,359,600–2,941,700 100% additional benefit not proven
Vedolizumab (1) Entyvio® Takeda Pharma Vertrieb GmbH & Co. KG Digestive system diseases Ulcerative colitis, Crohn's disease 16,100 100% additional benefit not proven
Fingolimod (2) Gilenya® Novartis Pharma GmbH Nervous system diseases Multiple sclerosis (MS), pre-treated 0
8,240–8,390 		100% additional benefit not proven repealed
Siltuximab (1) Sylvant® Janssen-Cilag GmbH Oncological diseases Multicentric Castleman Disease (MCD) 130–1,460 100% non-quantifiable additional benefit Orphan
Insulin degludec (2) Tresiba® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 2, combination with GLP-1 agonists 0
170,100 		100% additional benefit not proven repealed
Simeprevir (1) Olysio® Janssen-Cilag GmbH Infectious diseases Chronic hepatitis C 64,800 91% Indication of considerable additional benefit
Elosulfase alfa (1) Vimizim® BioMarin Deutschland GmbH Metabolic diseases Mucopolysaccharidosis (type IVA) 0
15–95 		100% minor additional benefit Orphan repealed
Mirabegron (1) Betmiga® Astellas Pharma GmbH Genitourinary system diseases Imperative desire to urinate 7,422,000 100% additional benefit not proven
Lebende Larven von Lucilia sericata (1) BioBag, BioMonde Freie Larven® BioMonde GmbH Other diseases Chronic or hard to heal wounds 500,000–1,000,000 100% additional benefit not proven
Perampanel (2) Fycompa® Eisai GmbH Nervous system diseases Epilepsy, focal seizures, ≥ 12 years 70,600–106,800 100% additional benefit not proven
Cholsäure (1) Orphacol® Laboratoires CTRS Metabolic diseases Bile acid synthesis disorder 10–25 100% non-quantifiable additional benefit Orphan
Ruxolitinib (2) Jakavi® Novartis Pharma GmbH Oncological diseases Myelofibrosis (MF) 1,600–5,000 100% Hint for considerable additional benefit Orphan (turnover limit)
Dimethylfumarat (1) Tecfidera® Biogen Idec GmbH Nervous system diseases Relapsing-remitting multiple sclerosis (MS) 85,000–105,000 100% additional benefit not proven
Insulin degludec (1) Tresiba® Novo Nordisk Pharma GmbH Metabolic diseases Diabetes mellitus type 1 and type 2 161,750
1,095,950 		100% additional benefit not proven repealed subpopulations
Riociguat (1) Adempas® Bayer Vital GmbH Cardiovascular diseases Pulmonary arterial hypertension (PAH), Chronic thromboembolic pulmonary hypertension (CTPH) 0
1,500–13,310 		100% minor additional benefit Orphan repealed
Cobicistat (1) Tybost® Gilead Sciences GmbH Infectious diseases HIV infection 57,400 100% additional benefit not proven
Canagliflozin (1) Invokana® Janssen-Cilag GmbH Metabolic diseases Diabetes mellitus type 2 1,253,400–1,453,400 100% additional benefit not proven
Bedaquilin (1) Sirturo® Janssen-Cilag GmbH Infectious diseases Multidrug-resistant pulmonary tuberculosis n.d. discontinued Orphan
Dapagliflozin / Metformin (1) Xigduo® Bristol-Myers Squibb GmbH & Co. KGaA/ AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2 0
615,300 		100% additional benefit not proven repealed
Dolutegravir (1) Tivicay® ViiV Healthcare GmbH Infectious diseases HIV infection, ≥ 12 years 57,399 11% Proof of considerable additional benefit
Olodaterol (1) Striverdi Respimat® Boehringer Ingelheim Pharma GmbH & Co. KG Respiratory system diseases Chronic obstructive pulmonary disease (COPD) n.d. 100% additional benefit not proven
Sofosbuvir (1) Sovaldi® Gilead Sciences GmbH Infectious diseases Chronic hepatitis C 99,841 4% Indication of considerable additional benefit
Macitentan (1) Opsumit® Actelion Pharmaceuticals Deutschland GmbH Cardiovascular diseases Pulmonary arterial hypertension (PAH) 0
580–7,850 		100% minor additional benefit Orphan repealed
Retigabin (3) Trobalt® GlaxoSmithKline GmbH & Co. KG Nervous system diseases Epilepsy, focal seizures 26,800–53,600 100% additional benefit not proven
Turoctocog alfa (1) NovoEight® Novo Nordisk Pharma GmbH Hematopoietic diseases Hemophilia A 3,190–3,585 100% additional benefit not proven
Trastuzumab Emtansin (1) Kadcyla® Roche Pharma AG Oncological diseases Breast cancer (BC) HER2+, pre-treated patients 4,121 72% Indication of considerable additional benefit
Radium-223-dichlorid (1) Xofigo® Bayer Vital GmbH Oncological diseases Prostate carcinoma (PC) 0
22,700 		78% Indication of considerable additional benefit repealed
Emtricitabin / Rilpivirin / Tenofovirdisoproxil (2) Eviplera® Gilead Sciences GmbH Infectious diseases HIV infection 46,000 100% additional benefit not proven
Lomitapid (1) Lojuxta® Aegerion Pharmaceuticals GmbH Metabolic diseases Hypercholesterolemia 0
61–71 		100% additional benefit not proven repealed
Ipilimumab (2) Yervoy® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, first-line 500–1,500 100% additional benefit not proven
Afatinib (1) Giotrif® Boehringer Ingelheim Pharma GmbH & Co. KG Oncological diseases Non-small cell lung carcinoma (NSCLC), EGFR mutation, EGFR-TKI-naive patients 0
640–4,980 		45% Indication of considerable additional benefit repealed
Indacaterol / Glycopyrronium (1) Ultibro Breezhaler, Xoterna Breezhaler® Novartis Pharma GmbH Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 2,359,628–2,949,660 5% Indication of minor additional benefit
Saxagliptin (2) Onglyza® Bristol-Myers Squibb GmbH & Co. KGaA und AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2, monotherapy n.d. discontinued
Rivaroxaban (1) Xarelto® Bayer Vital GmbH Hematopoietic diseases Prophylaxis / therapy of artherotrombotic / venothrombotic events n.d. discontinued
Denosumab (1) Prolia, XGEVA® Amgen GmbH Musculoskeletal system diseases Osteoporosis (postmenopausal), bone loss (prostate cancer) n.d. discontinued
Dabigatranetexilat (1) Pradaxa® Boehringer Ingelheim Pharma GmbH & Co. KG Hematopoietic diseases Prevention of venous thromboembolic events after hip or knee replacement and prevention of stroke and systemic embolism n.d. discontinued
Dabrafenib (1) Tafinlar® GlaxoSmithKline GmbH & Co. KG Oncological diseases Melanoma, BRAF V600 mutation 1,400 100% additional benefit not proven
Teriflunomid (1) Aubagio® Sanofi-aventis groupe/Genzyme GmbH Nervous system diseases Relapsing-remitting multiple sclerosis (MS) 85,000–105,000 100% additional benefit not proven
Regorafenib (1) Stivarga® Bayer Vital GmbH Oncological diseases Colorectal carcinoma (CRC) 0
6,600–14,000 		100% Hint for minor additional benefit repealed
Aflibercept (2) Eylea® Bayer Vital GmbH Eye diseases Macular edema following retinal vein occlusion 19,600–21,200 100% additional benefit not proven
Fluticasonfuroat / Vilanterol-Trifenatat (1) Relvar Ellipta® GlaxoSmithKline GmbH & Co. KG Respiratory system diseases Bronchial asthma, COPD n.d. 100% additional benefit not proven
Vemurafenib (2) Zelboraf® Roche Pharma AG Oncological diseases Melanoma, BRAF V600 mutation 1,400 100% Indication of considerable additional benefit
Pomalidomid (1) Imnovid® Celgene GmbH Oncological diseases Multiple myeloma (MM), at least 2 prior therapies, combination with dexamethasone 0
1,900 		100% considerable additional benefit Orphan repealed
Enzalutamid (1) Xtandi® Astellas Pharma GmbH Oncological diseases Prostate carcinoma (PC), progression during or after docetaxel-containing chemotherapy 6,300 100% Indication of considerable additional benefit
Vismodegib (1) Erivedge® Roche Pharma AG Oncological diseases Basal cell carcinoma (BCC) 0
295 		95% Hint for minor additional benefit repealed
Ponatinib (1) Iclusig® ARIAD Pharmaceuticals (Germany) GmbH Oncological diseases Chronic myeloid leukaemia (CML); acute lymphoblastic leukaemia (ALL), Ph+ or T315I mutation 0
525–1,135 		100% non-quantifiable additional benefit Orphan repealed
Nepafenac (1) Nevanac® Alcon Pharma GmbH Eye diseases Postoperative pain after eye surgery 850,000–1,000,000 100% additional benefit not proven
Defibrotid (1) Defitelio® Gentium SpA Other diseases Severe hepatic veno-occlusive disease in haematopoietic stem cell transplantation n.d. exempted Orphan
Elvitegravir / Cobicistat / Emtricitabin / Tenofovirdisoproxil (1) Stribild® Gilead Sciences GmbH Infectious diseases HIV infection 49,800 100% additional benefit not proven
Parathyroidhormon (1) Preotact® Takeda GmbH Musculoskeletal system diseases Osteoporosis (postmenopausal) n.d. discontinued
Lisdexamfetamindimesilat (1) Elvanse® Shire Deutschland GmbH Mental illnesses Attention deficit hyperactivity disorder (ADHD), ≥ 6 to < 18 29,600–59,600 100% additional benefit not proven
Ocriplasmin (1) Jetrea® ThromboGenics NV / Alcon Pharma GmbH Eye diseases Vitreomacular traction (VMT) 0
9,970–39,300 		95% Hint for considerable additional benefit repealed
Linaclotid (1) Constella® Almirall Hermal GmbH Digestive system diseases Irritable bowel syndrome (IBS) 394,000–838,000 100% additional benefit not proven
Bosutinib (1) Bosulif® Pfizer Pharma GmbH Oncological diseases Chronic myeloid leukaemia (CML) 0
380–500 		100% non-quantifiable additional benefit Orphan repealed
Vildagliptin (1) Galvus, Jalra, Xiliarx® Novartis Pharma GmbH Metabolic diseases Diabetes mellitus type 2 1,669,500–1,869,500
1,705,400–1,905,400 		100% additional benefit not proven repealed subpopulations
Vildagliptin / Metformin (1) Eucreas, Icandra, Zomarist® Novartis Pharma GmbH Metabolic diseases Diabetes mellitus type 2 792,050–811,850 100% additional benefit not proven
Sitagliptin / Metformin (1) Janumet, Velmetia® MSD SHARP & DOHME GmbH Metabolic diseases Diabetes mellitus type 2 0
792,050–810,850 		78% Hint for minor additional benefit repealed
Sitagliptin (1) Januvia, Xelevia® MSD SHARP & DOHME GmbH Metabolic diseases Diabetes mellitus type 2 0
1,705,400–1,905,400 		64% Hint for minor additional benefit repealed
Saxagliptin / Metformin (2) Komboglyze® Bristol-Myers Squibb GmbH & Co. KGaA/ AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2, combination with sulphonylurea 62,400 100% additional benefit not proven
Saxagliptin (1) Onglyza® Bristol-Myers Squibb GmbH & Co. KGaA / AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2, combination therapy 0
1,182,900–1,382,900 		50% Hint for minor additional benefit repealed
Pertuzumab (1) Perjeta® Roche Pharma AG Oncological diseases Breast cancer (BC) HER2+, combination with trastuzumab and docetaxel 3,300–5,118 33% Hint for considerable additional benefit
Colestilan (1) BindRen® Mitsubishi Pharma Deutschland GmbH Other diseases Hyperphosphatemia 44,500–57,000 100% additional benefit not proven
Chloroprocain (1) Ampres® Meduna Arzneimittel GmbH Other diseases Spinal anaesthesia n.d. exempted
Vandetanib (2) Caprelsa® AstraZeneca GmbH Oncological diseases Thyroid carcinoma (MTC) 0
60–1,500 		100% Hint for minor additional benefit repealed
Lixisenatid (1) Lyxumia® Sanofi-Aventis Deutschland GmbH Metabolic diseases Diabetes mellitus type 2 903,000 100% additional benefit not proven
Retigabin (2) Trobalt® GlaxoSmithKline GmbH & Co. KG Nervous system diseases Epilepsy, focal seizures n.d. discontinued
Aflibercept (Zaltrap, 1) Zaltrap® Sanofi-Aventis Deutschland GmbH Oncological diseases Colorectal carcinoma (CRC) 3,500–10,400 100% Indication of minor additional benefit
Ingenolmebutat (1) Picato® LEO Pharma GmbH Skin diseases Actinic keratosis (AK) 0
1,112,000–3,253,000 		100% additional benefit not proven repealed
Fidaxomicin (1) Dificlir® Astellas Pharma GmbH Infectious diseases Clostridium difficile infections 33,300 41% Proof of considerable additional benefit
Abirateronacetat (2) Zytiga® Janssen-Cilag GmbH Oncological diseases Prostate carcinoma (PC), after androgen deprivation therapy, no indication for chemotherapy 15,000–28,800 100% Indication of considerable additional benefit
Apixaban (2) Eliquis® Bristol-Myers Squibb GmbH & Co. KGaA / Pfizer Deutschland GmbH Hematopoietic diseases Prophylaxis of strokes and systemic embolisms 926,000–1,093,000 100% Indication of minor additional benefit
Dapagliflozin (1) Forxiga® Bristol-Myers Squibb GmbH & Co. KGaA/ AstraZeneca GmbH Metabolic diseases Diabetes mellitus type 2 0
896,100 		100% additional benefit not proven repealed
Aflibercept (1) Eylea® Bayer Vital GmbH Eye diseases Neovascular age-related macular degeneration 305,000 100% additional benefit not proven
Pixantron (1) Pixuvri® CTI Life Sciences Ltd. Oncological diseases B-Cell Non-Hodgkin Lymphoma (NHL) 970 100% additional benefit not proven
Linagliptin (3) Trajenta® Boehringer Ingelheim International GmbH Metabolic diseases Diabetes mellitus type 2 450,000–650,000 100% additional benefit not proven
Brentuximab Vedotin (1) Adcetris® Takeda Pharma Vertrieb GmbH & Co. KG Oncological diseases Hodgkin lymphoma (HL), CD30+; Systemic anaplastic large cell lymphoma 75–420 100% non-quantifiable additional benefit Orphan
Saxagliptin / Metformin (1) Komboglyze® Astra Zeneca GmbH & Bristol-Myers Squibb GmbH CO. KG Metabolic diseases Diabetes mellitus type 2 0
729,650–748,450 		85% Hint for minor additional benefit repealed
Decitabin (1) Dacogen® Janssen-Cilag GmbH Oncological diseases Acute myeloid leukaemia (AML) 300–780 100% minor additional benefit Orphan
Crizotinib (1) Xalkori® Pfizer Pharma GmbH Oncological diseases Non-small cell lung carcinoma (NSCLC), ALK+, pre-treated 0
480 		71% Hint for considerable additional benefit repealed
Axitinib (1) Inlyta® Pfizer Pharma GmbH Oncological diseases Renal cell carcinoma (RCC), after failure of sunitinib or a cytokine theryapy 0
920 		0.7% Indication of minor additional benefit repealed
Aclidiniumbromid (1) Eklira Genuair, Bretaris Genuair® Almirall Hermal GmbH Respiratory system diseases Chronic obstructive pulmonary disease (COPD) 0
2,400,000–2,800,000 		100% additional benefit not proven repealed
Ruxolitinib (1) Jakavi® Novartis Pharma GmbH Oncological diseases Myelofibrosis (MF) 0
1,600 		100% minor additional benefit Orphan repealed
Perampanel (1) Fycompa® Eisai GmbH Nervous system diseases Epilepsy, focal seizures, ≥ 12 years 0
70,600–106,800 		100% additional benefit not proven repealed
Linagliptin (2) Trajenta® Boehringer Ingelheim International GmbH Metabolic diseases Diabetes mellitus type 2 1,219,500 100% additional benefit not proven
Ivacaftor (1) Kalydeco® Vertex Pharmaceuticals GmbH Metabolic diseases Cystic fibrosis (CF), G551D mutation, ≥ 6 years 0
170 		84% considerable additional benefit Orphan repealed
Tegafur / Gimeracil / Oteracil (1) Teysuno® Nordic Pharma GmbH Oncological diseases Gastric carcinoma 10,500–12,000 100% additional benefit not proven
Pasireotid (1) Signifor® Novartis Pharma GmbH Metabolic diseases Pituitary dysfunction 160–360 100% minor additional benefit Orphan
Vemurafenib (1) Zelboraf® Roche Pharma AG Oncological diseases Melanoma, BRAF V600 mutation 0
1,400 		100% Indication of considerable additional benefit repealed
Vandetanib (1) Caprelsa® AstraZeneca GmbH Oncological diseases Thyroid carcinoma (MTC) 0
130–1,300 		100% additional benefit not proven repealed
Ipilimumab (1) Yervoy® Bristol-Myers Squibb GmbH & Co. KGaA Oncological diseases Melanoma, second-line 3,100 100% Indication of considerable additional benefit
Belimumab (1) Benlysta® GlaxoSmithKline GmbH & Co. KG Musculoskeletal system diseases Systemic lupus erythematosus 7,000 100% Indication of considerable additional benefit
Fampridin (1) Fampyra® Biogen Idec GmbH Nervous system diseases Multiple sclerosis (MS) 43,100–49,900 100% additional benefit not proven
Belatacept (1) Nulojix® Bristol-Myers Squibb GmbH & Co. KGaA Genitourinary system diseases Prophylaxis of graft rejection (GvHD) after kidney transplantation 0
2,945–3,385 		100% Indication of minor additional benefit repealed
Emtricitabin / Rilpivirin / Tenofovirdisoproxil (1) Eviplera® Gilead Sciences GmbH Infectious diseases HIV infection, non-pretreated patients 1,260 100% Proof of minor additional benefit
Rilpivirin (1) Edurant® Janssen-Cilag GmbH Infectious diseases HIV infection 1,260 100% Proof of minor additional benefit
Extrakt aus Cannabis Sativa (1) Sativex® Almirall Hermal GmbH Nervous system diseases Multiple sclerosis (MS), spasticity 0
13,400–38,500 		100% Hint for minor additional benefit repealed
Tafamidis (1) Vyndaqel® Pfizer Pharma GmbH Metabolic diseases Amyloid Neuropathy 0
40–104 		100% minor additional benefit Orphan repealed
Apixaban (1) Eliquis® Bristol-Myers Squibb GmbH & Co. KGaA / Pfizer Deutschland GmbH Hematopoietic diseases Prophylaxis of venous thromboembolism 390,000 58% Indication of minor additional benefit
Piperaquintetraphosphat / Dihydroartemisinin (1) Eurartesim® Sigma-tau Arzneimittel GmbH Infectious diseases Antimalarial n.d. exempted
Ceftarolinfosamil (1) Zinforo® AstraZeneca GmbH Infectious diseases Beta-lactam antibiotic n.d. exempted
Aliskiren / Amlodipin (1) Rasilamlo® Novartis Pharma GmbH Cardiovascular diseases Essential hypertension, add-on therapy 156,600–565,900 100% additional benefit not proven
Retigabin (1) Trobalt® GlaxoSmithKline GmbH & Co. KG Nervous system diseases Epilepsy, focal seizures 0
85,000–175,000 		100% additional benefit not proven repealed
Eribulin (1) Halaven® Eisai GmbH Oncological diseases Breast cancer (BC) after at least 2 chemotherapies 0
5,630–7,310 		80% Hint for minor additional benefit repealed
Collagenase aus Clostridium histolyticum (1) Xiapex® Pfizer Pharma GmbH Musculoskeletal system diseases Dupuytren's contracture 35,000 100% additional benefit not proven
Telaprevir (1) Incivo® Janssen Cilag GmbH Infectious diseases Chronic hepatitis C 46,000 100% Indication of non-quantifiable additional benefit
Regadenoson (1) Rapiscan® Rapidscan Pharma Solutions EU Ltd. Other diseases Myocardial perfusion imaging 41,000 100% additional benefit not proven
Fingolimod (1) Gilenya® Novartis Pharma GmbH Nervous system diseases Rapidly progressing relapsing-remitting multiple sclerosis (MS) 0
9,500–7,500 		18% Hint for minor additional benefit repealed
Cabazitaxel (1) Jevtana® Sanofi-Aventis Deutschland GmbH Oncological diseases Prostate carcinoma (PC), docetaxel pre-treatment, combination with prednisone or prednisolone 5,670–6,930 85% Indication of minor additional benefit
Linagliptin (1) Trajenta® Boehringer Ingelheim International GmbH Metabolic diseases Diabetes mellitus type 2 0
1,219,500 		100% additional benefit not proven repealed
Abirateronacetat (1) Zytiga® Janssen-Cilag GmbH Oncological diseases Prostate carcinoma (PC), progression during or after docetaxel-containing chemotherapy, combination with prednisone or prednisolone 5,670–6,930 85% Indication of considerable additional benefit
Azilsartan Medoxomil (1) Edarbi® Takeda Pharma GmbH Cardiovascular diseases Arterial hypertension n.d. 100% additional benefit not proven
Pirfenidon (1) Esbriet® InterMune Deutschland GmbH Respiratory system diseases Idiopathic pulmonary fibrosis 6,000 100% non-quantifiable additional benefit Orphan
Boceprevir (1) Victrelis® MSD SHARP & DOHME GmbH Infectious diseases Chronic hepatitis C 46,000 100% Indication of non-quantifiable additional benefit
Bromfenac (1) Yellox® Bausch & Lomb/Dr. Mann Pharma Eye diseases Postoperative inflammation of the eye 850,000–1,000,000 100% additional benefit not proven
Ticagrelor (1) Brilique® AstraZeneca GmbH Cardiovascular diseases Prevention of atherothrombotic events in acute coronary syndrome 220,000–269,000 82% Proof of considerable additional benefit
Dexmedetomidin (1) Dexdor® Firma Orion Corporation Other diseases Hypnotics and sedatives, parenteral n.d. exempted
Pitavastatin (1) Livazo® Merckle Recordati GmbH Metabolic diseases Primary hypercholesterolaemia, mixed dyslipidaemia n.d. 100% additional benefit not proven
start postponed Trastuzumab deruxtecan (8) Enhertu® Daiichi Sankyo Deutschland GmbH Oncological diseases Solid tumours, HER2+ (IHC3+), previously treated n.d. active procedure