CurrentUnitedHealthcarePolicy CSOH2026D0104.D

Evkeeza (Evinacumab-Dgnb) (for Ohio Only)

Coverage criteria and utilization management for Evkeeza (evinacumab-dgnb) for treatment of homozygous familial hypercholesterolemia (HoFH) for UnitedHealthcare Community Plan in Ohio, affecting providers authorizing/administering the drug and members in Ohio.

Policy Summary

PayerUnitedHealthcare

PolicyEvkeeza (Evinacumab-Dgnb) (for Ohio Only)

Policy CodePolicy CSOH2026D0104.D

Change TypeRevised coverage criteriacoding updates

Effective DateJan 1, 2026

Next Review Date

Key ActionSubmit prior authorization with documentation confirming HoFH diagnosis and required LDL-C and treatment history per criteria.

SourceLink

POLICY UPDATE CHANGES

Revised coverage criteria for initial therapy; added criterion to allow coverage when the provider attests addition of ezetimibe to current therapy would not lower LDL-C to goal.

Added ICD-10 diagnosis codes E78.010, E78.011, and E78.019; removed ICD-10 diagnosis code E78.01.

12 monthsinitial authorization duration maximum

12 monthsreauthorization duration maximum

Age ≥ 5 yearsFDA-indicated population

LDL-C > 400 mg/dLuntreated LDL-C threshold

15 mg/kg Q4w

Policy Summary

PayerUnitedHealthcare

PolicyEvkeeza (Evinacumab-Dgnb) (for Ohio Only)

Policy CodePolicy CSOH2026D0104.D

Change TypeRevised coverage criteriacoding updates

Effective DateJan 1, 2026

Next Review Date

Key ActionSubmit prior authorization with documentation confirming HoFH diagnosis and required LDL-C and treatment history per criteria.

SourceLink

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Coverage Criteria for Evkeeza (evinacumab-dgnb)

Initial Therapy (Medical Necessity)

Covered when ALL of the following are met for initial therapy:

Initial therapy main criteria: Diagnosis of homozygous familial hypercholesterolemia (HoFH) by, or in consultation with, a lipid specialist (e.g., cardiologist, endocrinologist, lipidologist); AND confirmation of HoFH by genetic criteria OR by specified clinical criteria (either documentation of biallelic pathogenic/likely pathogenic variants on different chromosomes at LDLR, APOB, PCSK9, or LDLRAP1, or ≥2 such variants at different loci; OR untreated LDL-C > 400 mg/dL PLUS either xanthoma before 10 years of age OR evidence of familial hypercholesterolemia in at least one parent); AND one of the following: patient is < 10 years of age OR patient has failed to achieve an LDL-C goal of < 55 mg/dL despite other therapies (see adjunct therapy requirements for details on maximally tolerated statin/ezetimibe and intolerance definitions); AND patient will continue other LDL-C–lowering therapies in combination with Evkeeza; AND Evkeeza will not be used in combination with lomitapide (Juxtapid); AND dosing per U.S. FDA–approved labeling; AND initial authorization limited to no more than 12 months.

Genetic confirmation options: biallelic pathogenic/likely pathogenic variants on different chromosomes at LDLR, APOB, PCSK9, or LDLRAP1, or ≥2 such variants at different loci.

Continuation Therapy (Reauthorization)

Covered when ALL of the following are met for continuation of therapy:

Continuation therapy criteria: Documentation of a positive clinical response to Evkeeza therapy; Evkeeza will not be used in combination with lomitapide (Juxtapid); Evkeeza dosing is in accordance with U.S. FDA–approved labeling; and reauthorization will be for no more than 12 months.

Adjunct Therapy Requirements and Statin/Ezetimibe Exceptions

Adjunct/step requirements and exceptions:

Adjunct therapy and intolerance exceptions: Evkeeza is to be used as adjunct to other LDL-C lowering therapies (e.g., maximally tolerated statin therapy, ezetimibe). If the patient cannot tolerate statin therapy, intolerance must be evidenced by one of the following persistent (>2 weeks) findings: myalgia (muscle symptoms without CK elevations), myositis (muscle symptoms with CK elevations < 10× ULN), or rhabdomyolysis/muscle symptoms with CK elevations > 10× ULN. If the patient has a contraindication to all statins, one of the following must also be met: patient is receiving ezetimibe as adjunct to maximally tolerated statin therapy; OR patient has history of intolerance or contraindication to ezetimibe AND provider attests that addition of ezetimibe to current therapy would not lower LDL-C to goal. Additionally, one of the following regarding PCSK9 therapy must be met: prior treatment with PCSK9 inhibitor with inadequate response; OR physician attestation of two LDL-receptor negative alleles predicting nonresponse to PCSK9 therapy; OR history of intolerance/contraindication to PCSK9 therapy. Prior treatment with lomitapide (Juxtapid) or prior lipoprotein apheresis are also recognized considerations.

Provider attestation regarding ezetimibe response is an added pathway per revision.

Use of Evkeeza (evinacumab-dgnb) for causes of hypercholesterolemia other than homozygous familial hypercholesterolemia (HoFH) is not established. The FDA label specifies that safety and effectiveness have not been established in patients with other causes of hypercholesterolemia, including heterozygous familial hypercholesterolemia (HeFH). Additionally, Evkeeza will not be used in combination with lomitapide (Juxtapid) as required by the policy’s step-therapy/combination rules and exclusions.

Indications for Evkeeza outside of HoFH are not established and therefore are outside the scope of this policy. The FDA states that safety and effectiveness have not been established for patients with other causes of hypercholesterolemia, including heterozygous familial hypercholesterolemia (HeFH), and the policy does not provide coverage pathways for those diagnoses.

Applicable Codes and Diagnostic Thresholds

Covered HCPCS/Procedure CodesHCPCSCovered

J1305

Injection, evinacumab-dgnb, 5 mg.

Diagnosis CodesICD-10

E78.010	Homozygous familial hypercholesterolemia [HoFH].
E78.011	Heterozygous familial hypercholesterolemia [HeFH].
E78.019	Familial hypercholesterolemia, unspecified.
Z83.42	Family History of FH

Untreated LDL-C threshold — indicative of HoFH

Untreated LDL-C thresholdLDL-C greater than 400 mg/dL

Source context — numeric equivalenceLDL-C > 10 mmol/L is equivalent to > 400 mg/dL (per professional society guidance)

Use in diagnostic pathwayThreshold is used as part of clinical criteria when genetic confirmation is not available (requires xanthoma before age 10 or familial evidence)

Prior Authorization, Documentation, and Operational Rules

Prior Authorization

Prior Authorization Required

Prior authorization is required for Evkeeza (evinacumab‑dgnb). Requests must document that the member meets initial or continuation criteria and will be limited to the specified authorization durations.

Prior authorization required for initial and continuation therapy
Initial authorization: up to 12 months
Reauthorization: up to 12 months

Step Therapy

Step Therapy / Combination Therapy Rules

Evkeeza is intended as an adjunct to other LDL‑C–lowering therapies. Step‑therapy and combination rules apply; exceptions permitted for documented intolerance or contraindication.

Evkeeza must be used in combination with other traditional LDL‑C lowering therapies (e.g., maximally tolerated statin therapy, ezetimibe) unless intolerance/contraindication is documented

Clinical Background and Scope

Homozygous familial hypercholesterolemia (HoFH) is a rare autosomal disorder characterized by markedly elevated low-density lipoprotein cholesterol (LDL-C) and premature cardiovascular disease. It results from bi-allelic pathogenic or likely pathogenic variants in genes such as LDLR, APOB, PCSK9, or LDLRAP1, and clinical criteria (for example, untreated LDL-C > 400 mg/dL with early xanthoma or parental evidence of familial hypercholesterolemia) may also support the diagnosis. Evkeeza (evinacumab-dgnb) is an adjunctive monoclonal antibody therapy that inhibits ANGPTL3 and is indicated for patients aged 5 years and older with HoFH when used alongside other LDL-C lowering therapies per FDA labeling.

Definitions and Drug Description

Homozygous familial hypercholesterolemia (HoFH) — diagnostic definition

Genetic confirmation (preferred)Biallelic pathogenic/likely pathogenic variants on different chromosomes at LDLR, APOB, PCSK9, or LDLRAP1, or ≥2 such variants at different loci

Clinical criteria (alternative when genetics unavailable)Untreated LDL-C > 400 mg/dL plus either xanthoma before 10 years of age or evidence of familial hypercholesterolemia in at least one parent

Specialist involvementDiagnosis should be made by, or in consultation with, a lipid specialist (e.g., cardiologist, endocrinologist, lipidologist)

Professional society alignmentEuropean Atherosclerosis Society guidance recognizes LDL-C >10 mmol/L (>400 mg/dL) and early xanthomas/familial history as suggestive of HoFH

Evinacumab-dgnb (Evkeeza) — mechanism and clinical effect