WHA
 

Juxtapid (lomitapide)

Indications for Prior Authorization

Juxtapid (lomitapide)
  • For diagnosis of Homozygous familial hypercholesterolemia (HoFH)
    Homozygous familial hypercholesterolemia (HoFH) indicated as an adjunct to a low-fat diet and exercise and other low density lipoprotein cholesterol (LDL-C) therapies to reduce LDL-C in adults and pediatric patients aged 2 years and older with homozygous familial hypercholesterolemia (HoFH).

Criteria

Juxtapid

Prior Authorization

Length of Approval: When approved; no reauthorization required

  • Diagnosis of homozygous familial hypercholesterolemia (HoFH) as confirmed by one of the following: [1-3]
    • 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:
      • Untreated LDL-C greater than 400 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 2 years of age or older
    • AND
  • One of the following:
    • Patient is receiving other lipid-lowering therapy (e.g., statin, ezetimibe) [A]
      • OR
    • Patient has an inability to take other lipid-lowering therapy (e.g., statin, ezetimibe)
    AND
  • One of the following:
    • Patient is less than 10 years of age
      • OR
    • Both of the following:
      • Patient is 10 years of age or older
        • AND
      • Trial and failure, contraindication or intolerance to Repatha therapy
    AND
  • Prescribed by or in consultation with one of the following:
    • Cardiologist
    • Endocrinologist
    • Hepatologist
    AND
  • Not used in combination with a proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor (e.g., Praluent, Repatha)

  • Guideline ID
    GL-577281

  • Formulary
    Premium

  • Effective date
    Jul 1, 2026

  • P&T approval date
    Feb 19, 2013

P & T Revisions

2026-05-08, 2025-12-18, 2025-11-06, 2025-05-01, 2025-04-30, 2025-03-24, 2024-11-07, 2023-08-31, 2022-10-21, 2022-06-09, 2021-08-30, 2020-09-03, 2019-11-15

  1. Juxtapid Prescribing Information. Aegerion Pharmaceuticals, Inc. Cambridge, MA. April 2026.
  2. Raal FJ, Santos RD. Homozygous familial hypercholesterolemia: current perspectives on diagnosis and treatment. Atherosclerosis. 2012;223:262-8.
  3. 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.
  4. 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.
  5. 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.
  6. American Board of Clinical Lipidology website. www.lipidboard.org. Accessed September 3, 2020.
  7. Accreditation Council for Clinical Lipidology website. www.lipidspecialist.org. Accessed September 3, 2020.
  8. 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-cholersterol lowering in the management of atherosclerotic cardiovascular disease risk. J Am Coll Cardiol. 2017;70:1785-1822.
  9. Cuchel M, Raal FJ, Hegele RA, et al. 2023 Update on European Atherosclerosis Society Consensus Statement on Homozygous Familial Hypercholesterolaemia: new treatments and clinical guidance. Eur Heart J. 2023;44(25):2277-2291. doi:10.1093/eurheartj/ehad197
  1. IMPROVE-IT was a prospective RCT evaluating the addition of ezetimibe to simvastatin 40 mg in a high-risk patient population for secondary prevention over 7 years. The addition of ezetimibe significantly reduced ASCVD events, albeit very modestly (HR 0.936; 95% CI 0.887, 0.988; p = 0.016; number needed to treat [NNT] = 50). [5] The effect of lomitapide on cardiovascular morbidity and mortality has not been determined. [1]
  2. Lipid specialists are physicians certified by the American Board of Clinical Lipidology (ABCL) or the Accreditation Council for Clinical Lipidology (ACCL). [6, 7] In the opinion of the ACC expert consensus writing committee, lomitapide is best administered under the care of a lipid specialist. [8]
  3. Per the 2018 ACC/AHA national treatment guidelines, adherence, response to therapy, and adverse effects should be monitored within 4 -12 weeks following LDL-C lowering medication initiation or dose adjustment, repeated every 3 to 12 months as needed. [4]
  • 2026-05-08: Criteria updated to include age indication as per PI.
  • 2025-12-18: no criteria changes, added IL statute operational note
  • 2025-11-06: 2025 Annual Review. No criteria changes. Updated references.
  • 2025-05-01: Approval length updated to "Approved- no reauthorization required"
  • 2025-04-30: Removing reauthorization requirement as part of extended reauthorization program.
  • 2025-03-24: Removing reauthorization requirement as part of extended reauthorization program.
  • 2024-11-07: 2024 Annual Review. Criteria update. Updated references.
  • 2023-08-31: Annual Review - no criteria changes
  • 2022-10-21: Annual Review - criteria updated
  • 2022-06-09: Removed submission of medical records and/or paid claims requirement from prior authorization criteria
  • 2021-08-30: Annual review: updated references, removed GPIs for discontinued strengths
  • 2020-09-03: Annual review; updated references.
  • 2019-11-15: Annual review; updated background and references.