JUXTAPID (lomitapide)

JUXTAPID (lomitapide) 

Self-Administration - oral

Diagnosis considered for coverage:

• Indicated as an adjunct to a low-fat diet and other lipid-lowering treatments, including LDL apheresis where available, to reduce low-density lipoprotein cholesterol (LDL-C), total cholesterol (TC), apolipoprotein B (apo B), and non-high-density lipoprotein cholesterol (non-HDL-C) in patients with homozygous familial hypercholesterolemia (HoFH).

Limitations of use:

• The safety and effectiveness of Juxtapid® have not been established in patients with hypercholesterolemia who do not have HoFH, including those with heterozygous familial hypercholesterolemia (HeFH).
• The effect of Juxtapid® on cardiovascular morbidity and mortality has not been determined.

 Coverage Criteria:

For diagnosis of Homozygous Familial Hypercholesterolemia (HoFH):

• Dose does not exceed 60 mg daily, AND
• Patient is 18 years of age or older, AND
• Prescribed by a cardiologist, endocrinologist, or lipid specialist, AND
• Submission of medical records (e.g., chart notes, laboratory values) documenting diagnosis of homozygous familial hypercholesterolemia (HoFH) as confirmed by one of the following:
  • Genetic confirmation of 2 mutations in the LDL receptor, ApoB, PCSK9, or LDL receptor adaptor protein 1 (i.e., LDLRAP1 or ARH), OR
  • Both of the following:
    • One of the following:
      • Untreated LDL-C greater than 500 mg/dL
      • Treated LDL-C greater than 300 mg/dL, AND
    • One of the following:
      • Xanthoma before 10 years of age
      • Evidence of heterozygous familial hypercholesterolemia (HeFH) in both parents, AND
• Patient is receiving other lipid-lowering therapy (e.g., statin, ezetimibe), AND
• Trial and failure, contraindication, or intolerance to Repatha® therapy, AND
• Patient has failed to achieve a low-density lipoprotein-cholesterol (LDL-C) goal of less than 100 mg/dL (or 70 mg/dL in HoFH patients with clinical cardiovascular disease (CVD)) (recent lab documentation required) while on maximally tolerated lipid-lowering therapy (e.g. statin, ezetimibe), AND
• Used as an adjunct to a low-fat diet and exercise regimen, AND
• Not used in combination with a proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor

Reauthorization Criteria:

For diagnosis of Homozygous Familial Hypercholesterolemia (HoFH):

• Dose does not exceed 60 mg daily, AND
• Prescribed by a cardiologist, endocrinologist, or lipid specialist, AND
• Submission of medical records (e.g., chart notes, laboratory values) documenting LDL-C reduction while on Juxtapid® therapy, AND
• Patient continues to receive other lipid-lowering therapy (e.g., statin, ezetimibe), AND
• Patient is continuing a low-fat diet and exercise regimen, AND
• Not used in combination with a proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor

Coverage Duration:

• Initial: 6 months
• Reauthorization: 1 year

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information:

• Contraindicated in the following conditions:
  • Pregnancy
  • Concomitant administration with moderate or strong CYP3A4 inhibitors, as this can increase Juxtapid® exposure
  • Patients with moderate or severe hepatic impairment (based on Child-Pugh category B or C) and patients with active liver disease, including unexplained persistent elevations of serum transaminases.

Policy Updates:

• 6/15/2021– Expanded criteria for diagnosis of HoFH, added contraindications; Coverage duration update
• 12/2/2013 – New policy approved by P&T.

References:

• Juxtapid Prescribing Information. Aegerion Pharmaceuticals, Inc. Cambridge, MA. December 2019.
• Raal FJ, Santos RD. Homozygous familial hypercholesterolemia: current perspectives on diagnosis and treatment. Atherosclerosis. 2012;223:262-8.
• Cuchel M, Bruckert E, Ginsberg HN, et al. Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society. Eur Heart J. 2014;35:2146-57.
• Grundy SM, Stone NJ, Bailey AL, et al. 2018 AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/APhA/ASPC/NLA/PCNA Guideline on the Management of Blood Cholesterol: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019; 73:e285-e350.
• Cannon CP, Blazing MA, Giugliano RP, et al. Ezetimibe added to statin therapy after acute coronary syndromes. N Engl J Med. 2015;372:2387-97.
• American Board of Clinical Lipidology website. www.lipidboard.org. Accessed September 3, 2020.
• Accreditation Council for Clinical Lipidology website. www.lipidspecialist.org. Accessed September 3, 2020.
• Lloyd-Jones DM, Morris PB, Ballantyne CM, et al. 2017 Focused Update of the 2016 ACC expert consensus decision pathway on the role of non-statin therapies for LDL-cholesterol lowering in the management of atherosclerotic cardiovascular disease risk. J Am Coll Cardiol. 2017;70:1785-1822.