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.

 


 

Last review date: June 15, 2021