CurrentCignaPolicy IP0128

Homozygous Familial Hypercholesterolemia - Evkeeza

Defines Cigna's prior authorization, coverage criteria, dosing, and exclusions for Evkeeza (evinacumab-dgnb IV) when used to treat homozygous familial hypercholesterolemia (HoFH). Affects providers requesting benefit coverage and authorization for Evkeeza for Cigna-administered plans.

Policy Summary

PayerCigna

PolicyHomozygous Familial Hypercholesterolemia - Evkeeza

Policy CodePolicy IP0128

Change TypeTitle and criteria clarifications

Effective Date

Next Review Date

Key ActionObtain prior authorization for Evkeeza; approvals are granted for 1 year when the patient meets the policy's Initial Therapy or Continuation criteria.

SourceLink

POLICY UPDATE CHANGES

Title changed from Evinacumab to Homozygous Familial Hypercholesterolemia - Evkeeza and multiple clarifications made to Initial Therapy vs Currently Receiving criteria, statin intolerance definition, diagnostic thresholds, parental criteria, and specialist requirement.

15 mg/kg q4wApproved dosing

1 yearInitial approval duration

>400 mg/dLUntreated LDL-C threshold

≥300 mg/dLTreated LDL-C threshold

J1305

Policy Summary

PayerCigna

PolicyHomozygous Familial Hypercholesterolemia - Evkeeza

Policy CodePolicy IP0128

Change TypeTitle and criteria clarifications

Effective Date

Next Review Date

Key ActionObtain prior authorization for Evkeeza; approvals are granted for 1 year when the patient meets the policy's Initial Therapy or Continuation criteria.

SourceLink

On This Page

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Coverage Criteria for Evkeeza (evinacumab-dgnb)

FDA-Approved Indication: Homozygous Familial Hypercholesterolemia

Approve for 1 year if the patient meets ONE of the following (A or B):

Overall pathways: Pathway A: Initial Therapy OR Pathway B: Currently Receiving Evkeeza

See children-specific branching

A) Initial Therapy

i. Diagnostic confirmation: Patient meets ONE of: (a) genetic testing confirming diagnosis; OR (b) untreated LDL-C > 400 mg/dL AND (1) clinical manifestations before age 10 OR (2) at least one parent had untreated LDL-C/total cholesterol consistent with familial hypercholesterolemia; OR (c) treated LDL-C ≥ 300 mg/dL
Genetic examples include pathogenic variants in LDLR, APOB, PCSK9, or LDLRAP1; 'untreated' refers to prior to therapy with any antihyperlipidemic agent
ii. Age and prior therapy: If age 1 to <10 years: patient meets age bucket; OR if ≥10 years: patient meets BOTH of the following: (1) either (a) tried a PCSK9 inhibitor for ≥8 continuous weeks with LDL-C after therapy ≥ 70 mg/dL OR (b) known to have two LDL-receptor negative alleles; AND (2) either (a) tried high-intensity statin and ezetimibe for ≥8 continuous weeks with LDL-C after therapy ≥ 70 mg/dL OR (b) statin intolerant per policy definition
Statin intolerance defined in policy (rhabdomyolysis or skeletal-muscle symptoms with atorvastatin and rosuvastatin that resolved on discontinuation)

B) Currently Receiving Evkeeza: Approve if patient is currently receiving Evkeeza and experienced a response to therapy (e.g., decreasing LDL-C, total cholesterol, non-HDL-C, or apolipoprotein B); if patient has not previously received Cigna approval for Evkeeza, Initial Therapy criteria must be met

Continuation requires evidence of response; restarting therapy requires meeting Initial Therapy criteria

Evkeeza (evinacumab-dgnb) is considered not medically necessary for treatment of heterozygous familial hypercholesterolemia (HeFH). The prescribing information and the policy state that the safety and effectiveness of Evkeeza have not been established in patients with hypercholesterolemia who do not have HoFH.

Use of Evkeeza for any non-HoFH indication — including but not limited to combined hyperlipidemia, primary (pure) hypercholesterolemia, dyslipidemia, or isolated/elevated LDL-C — is considered not medically necessary. The policy notes that the safety and efficacy of Evkeeza have not been established for these other forms of hyperlipidemia.

Billing and Coding

Considered Medically Necessary when criteria metHCPCSCovered

J1305

Injection, evinacumab-dgnb, 5 mg

inv-05: Untreated LDL-C diagnostic threshold supporting HoFH

Untreated LDL-C threshold> 400 mg/dL (untreated; prior to any antihyperlipidemic therapy)

Context for thresholdUsed as a diagnostic criterion for HoFH when combined with early clinical manifestations (before age 10) or at least one parent with untreated LDL-C/total cholesterol consistent with familial hypercholesterolemia

Source guidancePart of Initial Therapy diagnostic options (genetic testing OR untreated LDL-C > 400 mg/dL with supporting clinical/parental evidence)

inv-06: Treated LDL-C diagnostic threshold supporting HoFH

Treated LDL-C threshold≥ 300 mg/dL (treated)

Context for thresholdConsidered diagnostic for HoFH as one of the Initial Therapy options when documented after antihyperlipidemic therapy

Provider Requirements and Actions

Prior Authorization

Prior authorization required

Prior authorization is required for Evkeeza. Approvals are granted for 1 year when Initial or Continuation criteria are met. Requests for doses outside the dosing documented in the policy will be reviewed case-by-case by a clinician. Patients who previously met Initial Therapy criteria and are currently receiving Evkeeza need only meet Continuation criteria; patients restarting or who never met prior criteria must meet Initial Therapy criteria.

Step Therapy

Required prior lipid-lowering therapy for patients ≥10 years

For patients aged ≥10 years (and applicable older adolescents/adults), a trial of PCSK9 inhibitor therapy is required: examples include evolocumab (Repatha) or alirocumab (Praluent). The patient must have completed the required trial(s) (including at least 8 continuous weeks of high-intensity statin plus ezetimibe when indicated) and LDL‑C remains ≥ 70 mg/dL after this therapy. Alternatively, documented statin intolerance per policy criteria or presence of two LDL-receptor negative alleles may apply. Documentation of prior PCSK9 inhibitor use and post-treatment LDL-C must be provided.

Examples of PCSK9 inhibitors: Repatha (evolocumab), Praluent (alirocumab)
Required LDL-C threshold after PCSK9 therapy: ≥ 70 mg/dL

Definitions and Clinical Terms

inv-13: Definition of HoFH (Homozygous Familial Hypercholesterolemia)

Definition (summary)Homozygous Familial Hypercholesterolemia (HoFH) is a rare inherited disorder causing markedly elevated LDL‑C due to little to no LDL receptor function; prevalence ~1 in 300,000–1,000,000

Typical genetic causesOften due to biallelic pathogenic variants in LDLR; FH also associated with variants in APOB, PCSK9, or LDLRAP1 (examples cited)

Clinical implicationsPresents with markedly elevated LDL‑C, early tendon or cutaneous xanthomas (sometimes in childhood), and very high risk for premature atherosclerotic cardiovascular disease (ASCVD)

Diagnostic notesHoFH can be diagnosed by genetic testing or clinical criteria (e.g., untreated LDL‑C > 400 mg/dL with early xanthomas or parental LDL‑C consistent with FH)

inv-14: Statin intolerance (policy-defined)

Statin intolerance — major criterion

Background

Homozygous familial hypercholesterolemia (HoFH) is a rare inherited disorder characterized by markedly elevated LDL-C levels due to impaired LDL receptor function, often from biallelic pathogenic variants (for example in LDLR, APOB, PCSK9, or LDLRAP1). HoFH is associated with early tendon xanthomas and a high risk of premature atherosclerotic cardiovascular disease, and affected patients commonly require multiple LDL-C–lowering therapies (statins, ezetimibe, PCSK9 inhibitors, lomitapide, LDL apheresis) with adjunctive agents such as evinacumab considered when criteria are met.