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Juxtapid (lomitapide) — Medica

Homozygous Familial Hypercholesterolemia (HoFH)

Preferred products

  • Repatha (evolocumab)
  • Praluent (alirocumab)

Initial criteria

  • age ≥ 18 years
  • Diagnosis confirmed by ONE of the following (a, b, or c):
  • a) Genetic testing confirms HoFH with pathogenic variants in LDLR, APOB, PCSK9, or LDLRAP1 gene; OR
  • b) Untreated LDL-C > 400 mg/dL AND ONE of the following [(1) or (2)]: (1) clinical manifestations of homozygous familial hypercholesterolemia before age 10 years (cutaneous xanthomas, tendon xanthomas, arcus cornea, tuberous xanthomas, or xanthelasma); OR (2) at least one parent had untreated LDL-C ≥ 190 mg/dL and/or untreated total cholesterol > 250 mg/dL; OR
  • c) Treated LDL-C ≥ 300 mg/dL AND ONE of the following [(1) or (2)]: (1) clinical manifestations of homozygous familial hypercholesterolemia before age 10 years; OR (2) at least one parent had untreated LDL-C ≥ 190 mg/dL and/or total cholesterol > 250 mg/dL
  • Patient meets ONE of the following (a or b):
  • a) Tried at least one PCSK9 inhibitor (e.g., Repatha, Praluent) for ≥ 8 continuous weeks AND LDL-C after this therapy remains ≥ 70 mg/dL; OR
  • b) Patient is known to have two LDL-receptor negative alleles
  • Patient meets ONE of the following (a or b):
  • a) ALL of the following [(1), (2), and (3)]: (1) Tried one high-intensity statin (atorvastatin ≥ 40 mg daily or rosuvastatin ≥ 20 mg daily); AND (2) Tried one high-intensity statin along with ezetimibe for ≥ 8 continuous weeks; AND (3) LDL-C after this regimen remains ≥ 70 mg/dL; OR
  • b) Determined to be statin intolerant by ONE of the following [(1) or (2)]: (1) Experienced statin-related rhabdomyolysis (creatine kinase ≥ 10× ULN with end-organ damage including acute renal injury or myoglobinuria); OR (2) Experienced skeletal-related muscle symptoms (e.g., myopathy or myalgia) on separate trials of both atorvastatin and rosuvastatin that resolved upon discontinuation of each statin

Reauthorization criteria

  • Patient is currently receiving Juxtapid and, according to the prescriber, has experienced a response to therapy (e.g., decreasing LDL-C, total cholesterol, non-HDL-C, or apolipoprotein B levels)

Approval duration

1 year