CurrentCignaPolicy N/A

Juxtapid (lomitapide) prior authorization for Homozygous Familial Hypercholesterolemia

Defines prior authorization requirements, coverage criteria, and exclusions for Juxtapid (lomitapide) for treatment of adults with homozygous familial hypercholesterolemia (HoFH) for Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyJuxtapid (lomitapide) prior authorization for Homozygous Familial Hypercholesterolemia

Policy CodePolicy N/A

Change TypeMaterial revisions to initial and continuation criteria

Effective DateN/A

Next Review DateMay 8, 2024

Key ActionSubmit prior authorization showing age ≥18, phenotypic confirmation of HoFH or LDL‑C thresholds, required prior trials (PCSK9 inhibitor and high‑intensity statin ± ezetimibe) or documentation of statin intolerance.

SourceLink

POLICY UPDATE CHANGES

Statement that a patient who previously met initial therapy criteria and is currently receiving Juxtapid is only required to meet continuation of therapy criteria.

Initial therapy diagnostic requirements were revised: genetic confirmation requirement changed to 'phenotypic confirmation', LDL-C untreated threshold changed to >500 mg/dL to >400 mg/dL, parental requirement changed to at least one parent.

Distinction created between Initial Therapy criteria and criteria for patients currently receiving Juxtapid.

1 yearapproval duration for Juxtapid when criteria met

≥18minimum age for initial therapy approval

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Coverage Criteria for Juxtapid (lomitapide)

Initial Therapy and Continuation Therapy

Covered when ONE of the following is met (A or B). Approve for 1 year.

HoFH indication: A) Initial Therapy OR B) Patient Currently Receiving Juxtapid

A) Initial Therapy

i: Patient is ≥ 18 years of age>= 18 years
ii: Patient meets ONE of: (a) phenotypic confirmation of HoFH; OR (b) untreated LDL-C > 400 mg/dL AND (1) clinical manifestation before 10 years OR (2) at least one parent with untreated LDL-C or total cholesterol consistent with familial hypercholesterolemia; OR (c) treated LDL-C ≥ 300 mg/dL AND (1) clinical manifestation before 10 years OR (2) at least one parent with untreated LDL-C or total cholesterol consistent with familial hypercholesterolemia
iii: Patient meets ONE of: (a) tried ≥1 PCSK9 inhibitor for ≥8 continuous weeks AND LDL-C after this PCSK9 inhibitor therapy remains ≥70 mg/dL; OR (b) known to have two LDL-receptor negative allelesPCSK9 ≥8 weeks; post-PCSK9 LDL-C >=70 mg/dL
iv: Patient meets ONE of: (a) tried one high-intensity statin AND one high-intensity statin + ezetimibe for ≥8 continuous weeks with LDL-C after this regimen ≥70 mg/dL; OR (b) statin intolerant defined by (1) statin-related rhabdomyolysis OR (2) separate trials of atorvastatin and rosuvastatin causing skeletal muscle symptoms that resolved on discontinuationhigh-intensity statin doses as specified; trials >=8 weeks

B) Patient Currently Receiving Juxtapid: Approve if, per prescriber, the patient has experienced a response to therapy (examples: decreasing LDL-C, total cholesterol, non-HDL-C, or apolipoprotein B). If patient is currently receiving therapy but has not previously received approval through the Coverage Review Department, review under Initial Therapy criteria. If restarting therapy, Initial Therapy criteria must be met.response to therapy

Use of Juxtapid (lomitapide) for heterozygous familial hypercholesterolemia (HeFH) or for other forms of hyperlipidemia that are not due to homozygous familial hypercholesterolemia (HoFH) is considered experimental, investigational, or unproven and is excluded from coverage.

Per policy, Juxtapid is not medically necessary (NMN) for any indication other than treatment of HoFH; this includes use for HeFH and general hyperlipidemia where HoFH is not documented.

Key Clinical Thresholds and Trial Durations

LDL-C thresholds

Untreated LDL-C threshold> 400 mg/dL

Treated LDL-C threshold≥ 300 mg/dL

Post‑PCSK9 LDL-C threshold≥ 70 mg/dL

Trial durations and age

Minimum PCSK9 inhibitor trial duration≥ 8 continuous weeks

High‑intensity statin ± ezetimibe trial durationEach regimen ≥ 8 continuous weeks (high‑intensity statin alone and high‑intensity statin + ezetimibe)

Minimum patient age for therapy≥ 18 years

Prior Authorization, Documentation, and Step Therapy Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Juxtapid (lomitapide). Approvals are provided for 1 year when criteria are met. Patients currently receiving Juxtapid who previously met Initial Therapy criteria under the Coverage Review Department need only meet Continuation criteria (i.e., currently receiving therapy). If past criteria were not met under the Coverage Review Department and the patient is currently receiving Juxtapid, or is restarting Juxtapid, Initial Therapy criteria must be met.

Approve for 1 year when criteria are met
Current recipients with prior Coverage Review approval: continuation criteria only
Restarting or never-approved patients must meet Initial Therapy criteria

Step Therapy

Step Therapy and Statin/PCSK9 Requirements

For Initial therapy approval, the patient must meet the full HoFH clinical criteria and demonstrate prior trials of lipid-lowering therapy: either a trial of a PCSK9 inhibitor for ≥ 8 continuous weeks with LDL-C remaining ≥ 70 mg/dL after that trial, OR documented genetic evidence of two LDL-receptor negative alleles. In addition, patients must have tried high-intensity statin therapy and ezetimibe (statin + ezetimibe for ≥ 8 continuous weeks) with LDL-C remaining ≥ 70 mg/dL, unless the patient is statin intolerant as defined below.

Definitions and Clinical Confirmation

Phenotypic confirmation of HoFH

Phenotypic confirmation definitionClinical presentation consistent with HoFH, including pathogenic variants at LDLR, APOB, PCSK9, or LDLRAP1, or characteristic features such as untreated LDL‑C > 400 mg/dL, childhood tendon or cutaneous xanthomas, or parental lipid levels consistent with familial hypercholesterolemia

Genetic examples notedPathogenic variants may be found in LDLR, APOB, PCSK9, or LDLRAP1 genes

Characteristic clinical featuresUntreated LDL‑C > 400 mg/dL; tendon or cutaneous xanthomas (may occur in childhood); parental LDL‑C or total cholesterol consistent with familial FH

Background

Homozygous familial hypercholesterolemia (HoFH) is a rare, inherited disorder characterized by markedly elevated low-density lipoprotein cholesterol (LDL-C) due to severely impaired LDL receptor function. Prevalence is uncommon (approximately 1 in 300,000 to 1 in 1,000,000) and affected patients face a very high risk of premature atherosclerotic cardiovascular disease. Clinical features that support a diagnosis include markedly elevated untreated LDL-C (values such as > 400 mg/dL are suggestive), childhood tendon or cutaneous xanthomas, and a family history or parental lipid levels consistent with familial hypercholesterolemia. Management typically involves multiple lipid-lowering strategies — high-intensity statins, ezetimibe, PCSK9 inhibitors, LDL apheresis when indicated, and adjunctive agents such as lomitapide (Juxtapid) — to reduce LDL-C and mitigate cardiovascular risk.