Currentsierra health and lifePolicy 2026D0104J

Evkeeza

Defines medical necessity criteria, initial and continuation authorization requirements, applicable billing code(s), and clinical background for Evkeeza (evinacumab-dgnb) for treatment of HoFH within UnitedHealthcare commercial plans (may apply to Medicare Advantage in absence of NCD/LCD).

Policy Summary

Payersierra health and life

PolicyEvkeeza

Policy CodePolicy 2026D0104J

Change TypeRevised (codes removed) and clarified

Effective DateApr 1, 2026

Next Review Date

Key ActionProviders must submit documentation confirming HoFH diagnosis (genetic testing results or specified clinical criteria), prior and concurrent lipid-lowering therapies, statin/ezetimibe/PCSK9 status or intolerances, and lipid specialist consultation to obtain authorization; initial authorization limited to ≤12 months.

SourceLink

POLICY UPDATE CHANGES

Removed ICD-10 diagnosis codes E78.011, E78.019, and Z83.42 from Applicable Codes.

Updated supporting information and clinical evidence sections to reflect current information.

1Primary Indication (HoFH)

J1305HCPCS code listed

E78.010ICD-10 primary diagnosis code

12 monthsAuthorization period (initial/renewal)

Coverage Summary

Coverage stance: covered with criteria for Evkeeza (evinacumab-dgnb) for the treatment of homozygous familial hypercholesterolemia (HoFH). Scope: defines medical necessity criteria, initial and continuation authorization requirements, applicable billing code(s), and clinical background for Evkeeza (evinacumab-dgnb) for treatment of HoFH within UnitedHealthcare commercial plans (and may apply to Medicare Advantage in the absence of an NCD/LCD). Effective Date: 2026-04-01. Last review: 2026-04-01.

Quick Facts & Thresholds

Untreated LDL-C> 400 mg/dL

Treatment LDL-C goal (failure threshold)< 55 mg/dL (failure to achieve)

Authorization period12 months

Primary indicationHomozygous familial hypercholesterolemia (HoFH)

HCPCS codeJ1305

ICD-10 codeE78.010

Initial Therapy Criteria

Initial Therapy

Evkeeza is proven and medically necessary for HoFH patients who meet ALL of the following criteria:

Diagnosis and confirmation: Diagnosis of HoFH by, or in consultation with, a lipid specialist experienced in the management of HoFH
Confirmation of HoFH (one of)
- Genetic confirmation: Submission of medical records confirming genetic confirmation of biallelic pathogenic/likely pathogenic variants on different chromosomes at the LDLR, APOB, PCSK9, or LDLRAP1 genes or ≥ 2 such variants at different loci
- OR clinical criteria (both required)
  - LDL-C threshold:

Continuation Therapy Criteria

Positive clinical response: provider-documented clinical improvement in LDL-C or other relevant lipid parameters while on Evkeeza. For continuation/reauthorization, documentation of such a positive clinical response is required (e.g., recorded reductions in LDL-C or other lipid measures while receiving Evkeeza).

Unproven / Exclusions

Exclusions and not-covered combinations: Evkeeza must not be used in combination with Juxtapid (lomitapide); claims may be denied if Evkeeza is used with lomitapide. Other implied exclusions include use outside the HoFH indication or without the required documentation and lipid specialist consultation — absence of required diagnostic confirmation, prior/concurrent therapy documentation, or failure to follow FDA dosing labeling may lead to denial or noncoverage.

Applicable Codes

HCPCS Covered Drug CodeHCPCSCovered

J1305

Injection, evinacumab-dgnb, 5 mg

Diagnosis Code (Referenced)ICD-10Covered

E78.010

Homozygous familial hypercholesterolemia [HoFH]

Provider Actions & Authorization Requirements

Prior Authorization

Prior authorization required with documentation

Prior authorization required with documentation. Providers must submit documentation confirming HoFH diagnosis (genetic testing results or specified clinical criteria), prior and concurrent lipid-lowering therapies, statin/ezetimibe/PCSK9 status or intolerances, and lipid specialist consultation to obtain authorization. Initial authorization will be for no more than 12 months. Applicable codes: J1305; E78.010.

Genetic testing results or clinical criteria confirming HoFH (biallelic pathogenic/likely pathogenic variants on different chromosomes at LDLR, APOB, PCSK9, or LDLRAP1 genes or clinical criteria: untreated LDL-C > 400 mg/dL plus xanthoma before age 10 or family history).
History of prior and concurrent lipid-lowering therapies (e.g., statins, ezetimibe, PCSK9 inhibitors, lomitapide, apheresis).
Statin, ezetimibe, and PCSK9 status or documented intolerances/contraindications.
Documentation of lipid specialist involvement (diagnosis by or consultation with a lipid specialist).
Initial authorization limited to ≤12 months.

Clinical Evidence

Ages 5+Expanded pediatric approval supported by single-arm study NCT04233918 in 14 patients (ages 5–11)

Mechanism, indication, and safety/efficacy context: Evinacumab-dgnb is a recombinant human monoclonal antibody that binds and inhibits ANGPTL3, a regulator of lipoprotein metabolism, thereby lowering triglycerides and affecting LDL-C via enhanced VLDL processing and clearance. It is indicated as an adjunct to diet and other LDL-C lowering therapies in patients aged 1 year and older with HoFH. Clinical trial evidence (ELIPSE HoFH) demonstrated substantial LDL-C reductions (e.g., ~47% reduction versus placebo at 24 weeks in adults), supporting efficacy; pediatric evidence supports expanded approval in children aged ≥5 years in a single-arm study.

Background

Background: HoFH is a rare autosomal disorder characterized by markedly elevated LDL-C, premature cardiovascular disease, and often tendon/skin xanthomas; it results from two pathogenic mutations in causative genes (e.g., LDLR, APOB, PCSK9) leading to very high LDL-C and high cardiovascular risk. Evkeeza is a monoclonal antibody that inhibits ANGPTL3 and is indicated as an adjunct to diet and other LDL-C lowering therapies in patients aged 1 year and older with HoFH.

FDA pediatric expansion: The FDA has approved Evkeeza for use in adults and pediatric patients aged 1 year and older with HoFH, with an expanded pediatric indication supported by studies including a single-arm pediatric trial; for dosing details and specific administration instructions, refer to the U.S. FDA approved labeling (do not reproduce dosing tables here).

Definitions

Maximally tolerated statin therapyStatin therapy at the highest dose a patient can tolerate; includes documentation of intolerance if applicable (e.g., myalgia, myositis, rhabdomyolysis).

Positive clinical responseProvider-documented clinical improvement in LDL-C or other relevant lipid parameters while on Evkeeza (documentation required for continuation).

Revision History

04/01/2026material_changeLatest

Material: Archived previous policy version 2025D0104I and removed ICD-10 diagnosis codes E78.011, E78.019, and Z83.42 from Applicable Codes.

04/01/2026clarificationLatest

Non-material: Updated Clinical Evidence, FDA, and References/supporting information sections (administrative and content updates) to reflect current information; policy effective date listed as April 1, 2026.

Policy Summary

Payersierra health and life

PolicyEvkeeza

Policy CodePolicy 2026D0104J

Change TypeRevised (codes removed) and clarified

Effective DateApr 1, 2026

Next Review Date

SourceLink

On This Page

Untreated low-density lipoprotein cholesterol (LDL-C) greater than 400 mg/dL

One of

Xanthoma before 10 years of age
Family history: Evidence of familial hypercholesterolemia in at least one parent

Age or LDL-C response (one of)

Age < 10: Patient is less than 10 years of age
Patient ≥10 with LDL-C treatment failure (all of the following)
- Statin status (one of)
  - Maximally tolerated statin therapy: Patient is currently treated with maximally tolerated statin therapy
    Maximally tolerated defined per policy
  - Statin intolerance or contraindication (one of)
    Myalgia: Intolerable and persistent (> 2 weeks) myalgia (muscle symptoms without CK elevations)
    Myositis: Intolerable and persistent (> 2 weeks) myositis (muscle symptoms with CK elevations < 10× ULN)
    Rhabdomyolysis or muscle symptoms with statin treatment with CK elevations > 10× ULN
    Contraindication to all statins: Patient has a contraindication to all statins
- Ezetimibe status (one of)
  - Receiving ezetimibe: Patient has been receiving ezetimibe therapy as adjunct to maximally tolerated statin therapy
  - Ezetimibe intolerance: Patient has a history of intolerance or contraindication to ezetimibe
- PCSK9 / alternative therapy status (one of)
  - PCSK9 treated or nonresponse: Patient has been treated with PCSK9 therapy (e.g., alirocumab, evolocumab) or did not respond to PCSK9 therapy
  - LDL-receptor negative alleles: Physician attests patient is known to have two LDL-receptor negative alleles (little to no residual function) and therefore would not respond to PCSK9 therapy
  - PCSK9 intolerance: Patient has a history of intolerance or contraindication to PCSK9 therapy
  - Prior lomitapide: Patient has previously been treated with Juxtapid (lomitapide)
  - Prior apheresis:

Concurrent therapy and safety: Patient will continue other traditional LDL-C lowering therapies in combination with Evkeeza; Evkeeza will not be used in combination with Juxtapid (lomitapide); Evkeeza dosing is in accordance with US FDA approved labeling; initial authorization will be for no more than 12 months

Definitions relevant to initial criteria: Maximally tolerated statin therapy is statin therapy at the highest dose a patient can tolerate and should include documentation of intolerance if applicable (e.g., myalgia, myositis, rhabdomyolysis). The policy requires that the diagnosis of HoFH be by, or in consultation with, a lipid specialist (e.g., cardiologist, endocrinologist, lipid specialist/lipidologist) experienced in HoFH, and documentation such as genetic testing results or specified clinical criteria must be submitted. Note: Evkeeza dosing must be in accordance with the U.S. FDA approved labeling; consult the FDA label for dosing specifics.

Documentation Required

Continuation documentation

Continuation documentation. For reauthorization, provide documentation of a positive clinical response to Evkeeza therapy. Confirm Evkeeza is not being used in combination with lomitapide (Juxtapid) and that dosing is consistent with the U.S. FDA approved labeling. Reauthorization will be for no more than 12 months. Applicable code: J1305.

Documentation of positive clinical response (provider-documented improvement in LDL-C or other relevant lipid parameters).
Confirmation Evkeeza is not used with lomitapide (Juxtapid).
Dosing consistent with U.S. FDA approved labeling.
Reauthorization limited to ≤12 months.

Billing Rule

Dosing per FDA labeling

Dosing per FDA labeling. Evkeeza dosing must be in accordance with the U.S. FDA approved labeling for coverage and reimbursement; billing and claims for J1305 are subject to this requirement.

Follow U.S. FDA approved dosing for Evkeeza to ensure coverage and correct J1305 claim adjudication.

Denial Risk

Combination with lomitapide prohibited

Combination with lomitapide prohibited. Evkeeza will not be used in combination with Juxtapid (lomitapide); claims may be denied if Evkeeza is used in combination with lomitapide (Juxtapid).

Do not co-administer Evkeeza with lomitapide (Juxtapid); such combination may lead to claim denial.

Patient has previously been treated with lipoprotein apheresis