Evinacumab (2) – Evkeeza®

Homozygous familial hypercholesterolemia, ≥ 5 to < 12 years of age

Characteristics

Start date 15.01.2024 – Marketing authorisation: 11.12.2023
Resolution 04.07.2024
INN Evinacumab
Brand name Evkeeza®
Pharm. company Ultragenyx Germany GmbH
G-BA Procedure ID D-1028
ATC code C10AX17 Other lipid modifying agents (C10AX)
ICD-10 codes (AIS) E78.0Pure hypercholesterolemia, E78.2Mixed hyperlipidemia, E78.4Other hyperlipidemia, E78.5Hyperlipidemia, unspecified, E78.88, E78.9Disorder of lipoprotein metabolism, unspecified
Alpha-ID codes (AIS) I16606Hyperlipidemia, I16611Hyperlipoproteinuria, I2449Mixed hyperlipidemia, I2459Familial hyperlipidemia, I64600Primary hypercholesterolemia, I94846Dyslipidemia
Therapeutic area Metabolic diseases Hypercholesterolemia
Reason for procedure New therapeutic indication
Regulatory status Exceptional Circumstances
Specialty Bundling Combination therapy

Therapeutic indication of the resolution

Evkeeza is used as an adjuvant therapy to diet and other therapies to lower low density lipoprotein cholesterol (LDL-C) levels for the treatment of adolescents and children aged 5 to < 12 years with homozygous familial hypercholesterolemia (HoFH)

Subpopulation Indication Comparator
Children and adolescents aged 5 to < 12 years with homozygous familial hypercholesterolemia in whom dietary and drug options for lipid lowering have been exhausted - Evolocumab (from 10 years of age) possibly with concomitant lipid-lowering drug therapy, or – LDL apheresis (as an "ultima ratio" for therapy-refractory courses) possibly with concomitant lipid-lowering drug therapy, or – Evolocumab (from 10 years of age) and LDL apheresis (as a last resort for refractory courses), possibly with concomitant lipid-lowering drug therapy

Studies and Results

No. of studies
(best subpopulation)
0 (Data not accepted)
Study design
(best subpopulation)
Data not accepted (Dossier: Single-arm + no comparison)

  • Clinical trials
    • The pharmaceutical manufacturer has submitted the single-arm trial R1500-CL-17100 for the benefit assessment of evinacumab.

Children and adolescents aged 5 to < 12 years with homozygous familial hypercholesterolaemia, in whom dietary and pharmacological options for lowering lipid levels have been exhausted

  • The additional benefit is not proven.
  • To assess the additional benefit of evinacumab for the treatment of children and adolescents aged 5 to < 12 years with homozygous familial hypercholesterolaemia, in whom dietary and pharmacological options for lowering lipids have been exhausted, the pharmaceutical manufacturer cites the single-arm study R1500-CL-17100.
  • Due to the lack of a comparator, the single-arm study R1500-CL-17100 is not suitable for drawing conclusions regarding the additional benefit of evinacumab compared with the appropriate comparator therapy.

Courtesy translation only, please refer to the German original.

Associated procedures

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


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