Evkeeza (evinacumab-dgnb) — Medical Mutual

Initial criteria

• Patient is age ≥ 5 years; AND
• Baseline low-density lipoprotein cholesterol (LDL-C) labs have been obtained prior to initiating treatment; AND
• Patient does not have a diagnosis of heterozygous familial hypercholesterolemia (HeFH); AND
• Must be prescribed by, or in consultation with, a specialist in cardiology, lipidology, or endocrinology; AND
• Will not be used in combination with lomitapide; AND
• Patient has a confirmed diagnosis of Homozygous Familial Hypercholesterolemia (HoFH) by ANY of the following: Confirmed DNA test for functional mutation(s) in LDL receptor alleles or alleles known to affect LDL receptor functionality (LDLR, PCSK9, or apoB gene(s)); OR Untreated LDL-C > 500 mg/dL or treated LDL-C ≥ 300 mg/dL; AND (Cutaneous or tendon xanthoma before age 10 years; OR Untreated LDL-C levels in both parents consistent with HeFH); AND
• Must be used as an adjunct to a low-fat or heart-healthy diet; AND
• Patient has been receiving stable background lipid lowering therapy for at least 4 weeks; AND
• Therapy will be used in conjunction with other LDL-lowering therapies (e.g., statins, ezetimibe, PCSK9 inhibitors, LDL apheresis); AND
• Patient has tried and failed at least a 3 month trial of adherent therapy with ezetimibe used in combination with the highest available (or maximally tolerated) dose of atorvastatin OR rosuvastatin, unless contraindicated; AND
• Patient has tried and failed at least a 3 month trial of adherent combination therapy consisting of the highest available (or maximally tolerated) dose of atorvastatin OR rosuvastatin, ezetimibe, AND a PSCK9 inhibitor indicated for HoFH (e.g., evolocumab), unless contraindicated; AND
• Despite pharmacological treatment, unless contraindicated, with a PCSK9 inhibitor, statin, and ezetimibe, the patient’s LDL cholesterol ≥ 100 mg/dL (or ≥ 70 mg/dL for patients with clinical atherosclerotic cardiovascular disease [ASCVD]); AND
• If patient intolerant to maximum dose statin: Evidence of muscle-related side effects with statin use including: (1) Muscle symptoms resolve after statin discontinuation; AND (2) Muscle symptoms occurred when re-challenged; AND (3) Muscle symptoms occurred after switching to an alternative statin; AND (4) Non-statin causes of symptoms ruled out; OR
• Diagnosis of rhabdomyolysis associated with statin use, supported by acute neuromuscular illness or dark urine AND acute elevation in creatine kinase (usually > 5,000 IU/L or 5× ULN)

Reauthorization criteria

• Patient continues to meet universal and indication-specific criteria (except prerequisite therapy); AND
• Absence of unacceptable toxicity from therapy (e.g., severe hypersensitivity reactions); AND
• Patient has had a reduction in LDL-C compared to baseline labs; AND
• Patient continues to adhere to diet and background lipid lowering therapy (e.g., statin, ezetimibe, PCSK9 inhibitor, LDL apheresis)

Approval duration

3 months initial, 12 months renewal