CurrentUnitedHealthcarePolicy 2025 P 2072-13

Juxtapid (lomitapide) prior authorization

Defines prior authorization and medical necessity criteria for coverage of Juxtapid (lomitapide) for patients with homozygous familial hypercholesterolemia (HoFH); applies to UnitedHealthcare pharmacy benefit programs.

Policy Summary

PayerUnitedHealthcare

PolicyJuxtapid (lomitapide) prior authorization

Policy CodePolicy 2025 P 2072-13

Change TypeRevised diagnostic criteriaadded contraindication

Effective DateMay 1, 2025

Next Review DateFeb 1, 2025

Key ActionObtain prior authorization and submit genetic test results or documentation supporting HoFH diagnosis and baseline labs for approval.

SourceLink

POLICY UPDATE CHANGES

Revised HoFH diagnostic criteria to include more precise genetic terminology to account for genetic test result interpretation complexity as well as digenic mutations.

Added requirement that Juxtapid not be used in combination with Evkeeza (evinacumab-dgnb).

Updated diet requirement per label (low-fat diet) clarified in criteria.

Initial authorization period changed to 12 months.

12 monthsapproval period

HoFH onlyindication

>400 mg/dLLDL-C threshold

PCSK9 / Evkeeza

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Coverage Criteria

(untitled)

Covered when ALL of the following are met:

ALL of the following

Diagnosis of homozygous familial hypercholesterolemia (HoFH) confirmed by either genetic testing or clinical criteria.
Genetic confirmation = bi-allelic pathogenic/likely pathogenic variants on different chromosomes at LDLR, APOB, PCSK9, or LDLRAP1 genes or ≥2 such variants at different loci.
Genetic or Clinical Confirmation
- Genetic confirmation as described above.
- Clinical criteria (both required)
  - Untreated LDL-C > 400 mg/dL.
  - One of the following clinical features
    Xanthoma before 10 years of age.
    Evidence of familial hypercholesterolemia in at least one parent.
Patient is on a low-fat diet.
Juxtapid is an adjunct to a low-fat diet.
Patient is receiving other lipid-lowering therapy (e.g., statin, ezetimibe, LDL apheresis) where appropriate.
Document concurrent lipid-lowering treatments.
Prescriber type
- Prescribed by a cardiologist.
- Prescribed by an endocrinologist.
- Prescribed by a lipid specialist.
Prior intolerance/contraindication or current therapy
- Prior therapy requirements
  - History of intolerance, failure, or contraindication to PCSK9 inhibitor Repatha (evolocumab).
  - History of intolerance, failure, or contraindication to Evkeeza (evinacumab).
- Patient is currently receiving Juxtapid therapy.
Not used in combination with a PCSK9 inhibitor (e.g., Praluent [alirocumab], Repatha [evolocumab]).
Concurrent use with PCSK9 inhibitors is not permitted.
Not used in combination with Evkeeza (evinacumab).
Concurrent use with evinacumab is not permitted.

Reauthorization

Reauthorization covered when ALL of the following are met:

ALL of the following

Patient remains on a low-fat diet.
Ongoing adherence should be documented.
Patient continues to receive other lipid-lowering therapy as appropriate (e.g., statin, ezetimibe, LDL apheresis).
Document ongoing concurrent therapies.
Documentation of a positive clinical response to therapy from pre-treatment baseline (e.g., reduction in LDL-C or other lipid parameters).
Provide laboratory values comparing baseline and on-treatment results.
Prescriber type
- Prescribed by a cardiologist.

Juxtapid (lomitapide) is NOT supported for the treatment of hypercholesterolemia in patients who do not have homozygous familial hypercholesterolemia (HoFH), including heterozygous familial hypercholesterolemia (HeFH). The safety and efficacy of Juxtapid have not been established in these populations, and use outside the HoFH indication is not covered.

Juxtapid is indicated as an adjunct to a low-fat diet and other lipid-lowering treatments (including LDL apheresis where available) to reduce LDL-C, total cholesterol, apolipoprotein B, and non-HDL-C in patients with HoFH. The effect of Juxtapid on cardiovascular morbidity and mortality has not been determined.

Coding & Diagnostic Thresholds

LDL-C threshold

LDL-C thresholdUntreated LDL-C greater than 400 mg/dL

ContextUsed as the clinical diagnostic pathway threshold for HoFH when genetic confirmation is not available

Unitmg/dL

Provider Actions & Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required for Juxtapid (lomitapide). Initial and reauthorization approvals will be issued for 12 months when all clinical criteria below are met. Prior authorization may be granted via automated adjudication in some programs based on prior claim/medication history and diagnosis codes; however, manual review may be required if documentation is incomplete.

Authorization period: 12 months
Automated approvals: may occur based on prior claims/medication history and ICD-10 codes — program logic varies

Documentation Required

Requests must include medical records supporting the HoFH diagnosis and relevant baseline clinical data. Acceptable documentation includes genetic testing results demonstrating bi-allelic pathogenic/likely pathogenic variants consistent with HoFH (e.g., pathogenic/likely pathogenic variants in LDLR, APOB, PCSK9, LDLRAP1, or two such variants at different loci), or clinical documentation meeting HoFH diagnostic criteria (e.g., childhood xanthomas, family history). Include baseline lipid panel and evidence of current lipid-lowering therapies.

Background

Juxtapid (lomitapide) is a microsomal triglyceride transfer protein (MTP) inhibitor indicated as an adjunct to a low-fat diet and other lipid-lowering treatments (including LDL apheresis where available) to reduce LDL-C, total cholesterol, apolipoprotein B, and non-HDL-C in patients with homozygous familial hypercholesterolemia (HoFH). Per the policy, Juxtapid must be prescribed by an appropriate specialist (cardiologist, endocrinologist, or lipid specialist), used with other lipid-lowering therapies, and is not permitted to be combined with PCSK9 inhibitors or Evkeeza (evinacumab); authorizations are issued for 12 months when criteria are met.

Definitions

HoFH — Diagnostic criteria

Primary diagnostic confirmationGenetic confirmation of bi-allelic pathogenic/likely pathogenic variants in LDLR, APOB, PCSK9, or LDLRAP1 genes

Digenic allowanceTwo pathogenic/likely pathogenic variants at different loci (digenic) is permitted as diagnostic confirmation

Alternative clinical pathwayClinical criteria may be used if genetic confirmation unavailable (e.g., untreated LDL-C >400 mg/dL plus early xanthomas or family history)

Initial Therapy Criteria

inv-12: Initial Therapy — Initial therapy approval when diagnostic and concomitant therapy criteria met

Initial therapy approval when diagnostic and concomitant therapy criteria met

Initial therapy rules: See Coverage Criteria: genetic confirmation OR clinical criteria (LDL-C >400 mg/dL with early xanthomas or parental FH), patient on a low-fat diet, receiving other lipid-lowering therapy, prescribed by an appropriate specialist, and not combined with PCSK9 inhibitors or Evkeeza.

Continuation Criteria

inv-13: Continuation — Continuation allowed when criteria met or if patient currently on therapy

Continuation allowed when criteria met or if patient currently on therapy

Continuation: Patient currently on Juxtapid therapy may be approved to continue; reauthorization requires demonstration of clinical benefit from baseline and continuation of a low-fat diet and other lipid-lowering therapies; Juxtapid must not be used in combination with PCSK9 inhibitors or Evkeeza.

Step Therapy Requirements

Step	Requirement
Step 1	History of intolerance, failure, or contraindication to a PCSK9 inhibitor (e.g., Repatha [evolocumab]) prior to approval in specified diagnostic pathways.
Step 2	History of intolerance, failure, or contraindication to Evkeeza (evinacumab) prior to approval in specified diagnostic pathways.
Continuation allowance	Patient currently on Juxtapid therapy may continue; reauthorization requires documentation of positive clinical response and continued adherence to diet and concomitant lipid‑lowering therapies.
Disallowed combinations	Concurrent use of Juxtapid with PCSK9 inhibitors (e.g., Praluent, Repatha) or with Evkeeza (evinacumab-dgnb) is not permitted.

Quantity Limits

Juxtapid (lomitapide) — Quantity limits

Quantity limitsSupply limits may be in place for Juxtapid (lomitapide)

Program variabilitySupply-limit application may vary by program and therapeutic class due to automated approval processes

Authorization impactSupply limits do not preclude prior authorization requirement — plan may still require documentation for approval