CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Evkeeza (evinacumab-dgnb) coverage for HoFH

Policy governs coverage, authorization, dosing, and renewal criteria for Evkeeza (evinacumab-dgnb) for Medicaid, Commercial, and Medicare-Medicaid Plan members, primarily for treatment of homozygous familial hypercholesterolemia (HoFH). Affects prescribers, pharmacists, and prior authorization reviewers.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyEvkeeza (evinacumab-dgnb) coverage for HoFH

Policy CodePolicy N/A

Change TypeNo material change

Effective DateJul 1, 2021

Next Review DateN/A

Key ActionSubmit prior authorization with specialist verification, baseline labs, and documentation of required prior lipid‑lowering therapy trials.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

15 mg/kgrecommended IV dose once monthly

3 monthsinitial authorization length

1890 mgmaximum HCPCS units per 28 days

≥20%LDL-C reduction required for renewal

J1305billing code (per 5 mg)

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Coverage and Medical Necessity Criteria

Initial Approval (HoFH)

Covered when ALL of the following are met

Universal and eligibility: Patient is ≥5 years old; prescribed by or in consultation with a cardiology, lipidology, or endocrinology specialist; baseline LDL-C, total cholesterol, apoB, and non-HDL-C obtained prior to initiation (these baseline labs are required for renewal); patient does not have heterozygous familial hypercholesterolemia (HeFH)

Baseline labs required for renewal

HoFH diagnosis confirmation: Patient has confirmed HoFH by ONE of: untreated LDL-C >500 mg/dL or treated LDL-C ≥300 mg/dL; documented DNA test showing functional LDL receptor mutation(s) or alleles affecting LDL receptor function; cutaneous or tendon xanthoma before age 10; OR untreated LDL-C levels in both parents consistent with HeFH

Concomitant therapy and diet: Used as an adjunct to a low-fat or heart-healthy diet and in conjunction with other LDL-lowering therapies (statins, ezetimibe, PCSK9 inhibitors, LDL apheresis); patient must have been on stable background lipid-lowering therapy for ≥4 weeks

Evkeeza must not be used in combination with lomitapide (Juxtapid)

Prerequisite pharmacotherapy trials: Patient tried and failed at least a 3-month trial of adherent therapy with ezetimibe plus the highest available (or maximally tolerated) dose of atorvastatin OR rosuvastatin, unless contraindicated; AND tried and failed at least a 3-month trial of combination therapy consisting of the highest available (or maximally tolerated) dose of atorvastatin OR rosuvastatin, ezetimibe, AND a PCSK9 inhibitor indicated for HoFH (e.g., evolocumab, alirocumab), unless contraindicated

If statin intolerance is claimed, documentation of causal relationship and re-challenge details required

LDL threshold despite therapy: Despite pharmacologic treatment with a PCSK9 inhibitor, statin, and ezetimibe, patient has LDL-C ≥100 mg/dL (or >70 mg/dL for patients with clinical ASCVD)

Renewal Criteria

Renewal covered when ALL of the following are met

Continuation criteria: Patient continues to meet universal and indication-specific criteria (including concomitant requirements and stable background therapies); absence of unacceptable toxicity to therapy (e.g., severe hypersensitivity); patient has had a >20% reduction in LDL-C from the initial baseline labs; and patient continues adherence to diet and background lipid-lowering therapies>20% LDL-C reduction

Evkeeza must not be used in combination with lomitapide (Juxtapid)

General coverage criteria

Covered when use aligns with FDA-approved labeling and the plan's clinical criteria, with preference for cost-effective alternatives where appropriate.

Primary requirements: Prescriber must submit information demonstrating the indication is consistent with FDA labeling or relevant clinical literature; the plan may require trial of more cost-effective alternatives when appropriate; for INTEGRITY (Medicare-Medicaid Plan) members these criteria apply only in the absence of an applicable CMS NCD or LCD; Neighborhood will give individual consideration to each request based on prescriber-submitted information and other information available to the plan

Coverage decisions follow FDA labeling, relevant literature, and applicable NCD/LCD where required

Evkeeza (evinacumab) must not be prescribed in combination with Juxtapid (lomitapide). This prohibition is specified in the coverage criteria for both initial approval and renewal and applies whether used with other background lipid‑lowering therapies or diet.

Within the policy rationale section there are no additional explicit exclusions listed beyond those called out in the clinical criteria; coverage decisions are guided by FDA labeling, relevant clinical literature, and the plan’s written criteria rather than by a separate exclusion list.

Use of Evkeeza for heterozygous familial hypercholesterolemia (HeFH) is excluded by this policy: patients must have a confirmed diagnosis of homozygous familial hypercholesterolemia (HoFH) to meet the coverage criteria.

Denials may occur when submitted documentation does not demonstrate that use is consistent with FDA labeling or the plan’s clinical criteria; the plan reviews each prior authorization request and gives individual consideration based on the prescriber‑submitted information and other available data.

Billing, Codes, and Dose Limits

Billing/CPT/HCPCSHCPCS

J1305

Injection, evinacumab-dgnb, 5 mg

NDCNDC

61755-0013-xx	Evkeeza 345 mg/2.3 mL single-dose vial (NDC format)
61755-0010-xx	Evkeeza 1200 mg/8 mL single-dose vial (NDC format)

Covered Diagnosis CodesICD-10Covered

E78.00	Pure hypercholesterolemia, unspecified
E78.01	Familial hypercholesterolemia

Affected codes (not enumerated)mixed

affected codes

placeholder for codes affected by prior authorization (document references affected codes without listing)

Maximum dose per 28 days

Maximum dose per 28 days1890 mg every 28 days

HCPCS unit guidanceMax units listed as 'per dose and over time' with 1890 mg/28 days

Vial utilization noteVial strengths should be utilized to minimize drug waste

Renewal LDL-C reduction threshold

Renewal LDL-C reduction thresholdPatient must have >20% reduction in LDL-C from baseline for renewal

Continued background therapyPatient must continue adherence to diet and background lipid-lowering therapies (statin, ezetimibe, PCSK9 inhibitor, or LDL apheresis)

Prior Authorization, Step Therapy, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for Evkeeza (evinacumab-dgnb). Prescriptions must be submitted for review prior to initiation. For Medicare-Medicaid Plan (MMP) members who have received this medication within the past 365 days, therapy requirements may be waived but prior authorization is still required.

Effective Date: 2021-07-01
Prescriber specialty: Prescriber should be a specialist or in consultation with one
Contact: 1-800-963-1001

Denial Risk

Denial Risk if Criteria Not Met

Requests that do not demonstrate that the patient meets the plan's clinical criteria or FDA labeling (including age, confirmed diagnosis of HoFH, required baseline labs, or adherence to required prior/maximally tolerated therapies) may be denied. The plan will individually review prescriber-submitted information and other available data to determine coverage.

Key Definitions and Dosing

Homozygous Familial Hypercholesterolemia (HoFH) — diagnostic thresholds and confirmation criteria

Untreated/treatment LDL-C thresholdsHoFH may be confirmed by untreated LDL-C >500 mg/dL or treated LDL-C ≥300 mg/dL

Genetic confirmationDocumented DNA test showing functional mutation(s) in LDL receptor alleles or alleles known to affect LDL receptor functionality

Clinical signsCutaneous or tendon xanthoma before age 10 years

Parental lipid levelsUntreated LDL-C levels in both parents consistent with heterozygous FH can confirm diagnosis

Adjunct requirementsMust be used as an adjunct to a low-fat/heart-healthy diet and with stable background lipid-lowering therapy ≥4 weeks

Evkeeza dosing

Clinical Background

Evkeeza (evinacumab‑dgnb) is an angiopoietin‑like 3 (ANGPTL3) inhibitor indicated as an adjunct to other LDL‑C lowering therapies for patients with homozygous familial hypercholesterolemia (HoFH). Clinical trial data referenced in the policy support substantial LDL‑C reductions in HoFH when Evkeeza is used on background lipid‑lowering therapy. The recommended administration is 15 mg/kg by IV infusion over 60 minutes once monthly, and the plan’s criteria require use as an adjunct to diet and other LDL‑lowering therapies with documented baseline lipid measurements prior to initiation.