Pharmacy Prior Authorization Criteria

Juxtapid (lomitapide)

Indications for Prior Authorization

Juxtapid (lomitapide)

• For diagnosis of Homozygous familial hypercholesterolemia (HoFH)
  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: (1) 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). (2) The effect of Juxtapid on cardiovascular morbidity and mortality has not been determined.

Criteria

Juxtapid

For initial authorization request, approve through 12/31/2039 For reauthorization request, bypass criteria review and approve through 12/31/2039

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
• 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
• 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

P & T Revisions

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

References

1. Juxtapid Prescribing Information. Aegerion Pharmaceuticals, Inc. Cambridge, MA. September 2020.
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

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End Notes

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]

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Revision History

• 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.

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