CurrentHealth NetPolicy N/A

Clinical Policy: Evinacumab-dgnb (Evkeeza) coverage criteria for homozygous familial hypercholesterolemia (HoFH)

Defines medical necessity criteria, prior authorization requirements, and continuation criteria for Evkeeza (evinacumab-dgnb) for members with HoFH across Health Net lines of business (Commercial, Medicaid). Applies to prescribers and pharmacy/medical benefit reviewers.

Policy Summary

PayerHealth Net

PolicyEvinacumab-dgnb (Evkeeza) coverage for HoFH

Policy CodePolicy N/A

Change TypeNo material change

Effective DateFeb 11, 2021

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation (weight in kg, recent LDL-C, genetic or clinical evidence of HoFH, prior therapies or intolerance) to support approval.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

15 mg/kg q4wmaximum dose allowed

12 monthsapproval duration

Commercial, Medicaidlines of business

Age ≥1 yrFDA age reference

345 mg/2.3 mLvial strength

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Coverage Criteria for Evkeeza (evinacumab-dgnb)

Initial Therapy — Covered when ALL of the following are met for diagnosis of Homozygous Familial Hypercholesterolemia (HoFH):

Covered when ALL of the following are met for diagnosis of Homozygous Familial Hypercholesterolemia (HoFH):

HoFH diagnosis: Diagnosis of HoFH confirmed by either: (a) genetic mutation consistent with HoFH (pathogenic/likely pathogenic variant in LDLR, PCSK9, APOB, or LDLRAP1) OR (b) clinical criteria such as untreated LDL-C ≥ 400 mg/dL; treated LDL-C ≥ 300 mg/dL or non-HDL-C ≥ 330 mg/dL; tendon or cutaneous xanthomas by age 10; or family history with a parent having LDL-C ≥ 190 mg/dL.see text

Supported by genetic testing or clinical documentation (labs, exam, family history)

Prescriber specialty: Prescribed by or in consultation with a cardiologist, endocrinologist, or lipid specialist.

Document specialty or consultation notes

Age and labeling considerations: Age consistent with FDA labeling and policy updates; document patient age in chart.age per FDA label

See policy updates for pediatric age indications

Concomitant statin therapy for patients ≥18 years: Evkeeza prescribed in conjunction with a statin at the maximally tolerated dose for members ≥18, unless statin therapy is contraindicated or the member is statin-intolerant.adherence documented ≥4 months if on statin

See Appendix D/E for intensity and contraindications; document adherence for the last 4 months

Statin intolerance or contraindication: For statin-intolerant members, documentation of intolerance is required (e.g., statin-associated muscle symptoms [SAMS] with resolution on discontinuation and recurrence on re-challenge, or intolerable SAMS with documented re-challenge/titration), or documentation that statin therapy is contraindicated per appendices.see text

Include re-challenge documentation when available

Prior use of ezetimibe and PCSK9 inhibitors (for members ≥18): Members ≥18 must have been adherent to ezetimibe concomitant with a maximally tolerated statin for at least 4 months unless contraindicated or intolerant; failure of an 8-week trial of Praluent may be required unless contraindicated (or a 3-month trial when required by coadministration context).8 weeks or 3 months per context

Prior authorization may be required for Praluent; document prior trials or intolerances

Dosing and weight documentation: Dose request must not exceed 15 mg/kg every 4 weeks and current body weight in kilograms must be documented for dosing calculations.<= 15 mg/kg q4w

If request is for dose increase, new dose must not exceed this limit

Continuation Therapy — Covered when ALL of the following are met for continued therapy:

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

Current therapy or prior approval: Member is currently receiving Evkeeza through the Centene benefit or has previously met initial approval criteria for Evkeeza.

Continuity of care considerations apply; include prior approval documentation if available

Adherence to statin if statin tolerant: If the member is statin-tolerant, documentation of adherence to a maximally tolerated statin is required for continued coverage.

See Appendix D for statin intensity definitions

Response to therapy: Member is responding to therapy as evidenced by laboratory results within the last 3 months showing a reduction in LDL-C since initiation of Evkeeza therapy.LDL-C reduction documented within 3 months

Approval duration for continued therapy: 12 months

Initial Therapy (HoFH) — Covered when ALL of the following are met (policy-level statements and dosing/administration):

Covered when ALL of the following are met (policy-level statements and dosing/administration):

HoFH indication: Member has a diagnosis of homozygous familial hypercholesterolemia (HoFH) for which evinacumab (Evkeeza) is indicated.

HoFH-specific dosing and documentation requirements apply

Dosing: Evinacumab (Evkeeza) administered intravenously at a dose of 15 mg/kg every 4 weeks.15 mg/kg q4w

Product single-dose vial strength: 345 mg/2.3 mL

Documentation of current body weight (kg) for dosing calculations and documentation of prior therapies and adherence (e.g., maximally tolerated statin and ezetimibe when applicable) must be provided.

Policy revisions explicitly require weight documentation for pediatric dosing and adherence documentation on reauthorization

Requests for use of Evkeeza for non‑FDA approved indications that are not specifically addressed in this policy are not authorized unless the provider supplies sufficient documentation of efficacy and safety in accordance with the applicable off‑label use policy: CP.CPA.09 for commercial members or CP.PMN.53 for Medicaid members.

Consider documented contraindications and clinical limitations when evaluating requests. The policy identifies moderate or severe hepatic impairment (Child‑Pugh classes B and C) as a contraindication for ezetimibe-related therapy and lists hypersensitivity events (for example, anaphylaxis, angioedema, rash, urticaria) as clinically significant concerns for statins/ezetimibe. Additional statin risk factors referenced include unexplained ALT elevations ≥3× upper limit of normal, active liver disease, older age (including ≥75 years), prior hemorrhagic stroke, concomitant interacting drugs, and other comorbidities that increase hepatic or renal risk.

Use of Evkeeza for indications that are not FDA‑approved and are not covered in this policy will be denied unless the request is supported by adequate off‑label use documentation consistent with the referenced off‑label policies (see CP.CPA.09 and CP.PMN.53 for required evidence and rationale).

Evaluate for a history of immune‑mediated hypersensitivity to the HMG‑CoA reductase inhibitor class when considering lipid therapies. The policy calls out documented immune‑mediated hypersensitivity (for example, immune reactions to statins) as a clinical situation that may contraindicate or alter the selection of lipid‑lowering agents and should be addressed in the clinical documentation supporting therapy decisions.

Coding, Dosing Limits, and Thresholds

Coding — general notemixed

NDC/HCPCS not listed

No specific billing codes listed in this document window

HCPCS codes referencedHCPCS

J1305

LDL-C thresholds — untreated and treated

Untreated LDL-C thresholdUntreated LDL-C ≥ 400 mg/dL

Treated LDL-C thresholdTreated LDL-C ≥ 300 mg/dL

Additional referenced thresholdLDL-C ≥ 130 mg/dL (pediatric leniency referenced)

Dose limit

Maximum dosing limitDo not exceed 15 mg/kg every 4 weeks

Weight documentation requirementDocumentation of member's current weight in kg is required for dosing calculations

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required. Provider must document that the member meets the initial approval criteria for Homozygous Familial Hypercholesterolemia (HoFH), including required genetic or clinical documentation, and submit supporting clinical information such as office chart notes and laboratory results.

Evkeeza (evinacumab) dosing for HoFH: 15 mg/kg IV every 4 weeks (HCPCS J1305).
Provider must submit documentation of member's current weight in kg for dosing calculations.
Approval duration: 12 months.

Documentation Required

Therapy Trials and Intolerance Documentation

For initial and continued therapy, providers must document trials of and adherence to maximally tolerated statin therapy (and ezetimibe when required) or document contraindication or intolerance per policy appendices. For members ≥18 years, adherence to maximally tolerated statin (and concomitant ezetimibe when specified) for at least the prior 4 months is required unless contraindicated or intolerant.

Background and Drug Information

Evinacumab‑dgnb (Evkeeza) is a human monoclonal antibody that targets angiopoietin‑like 3 and is FDA‑approved as an adjunct to diet and other lipid‑lowering therapies to reduce LDL‑C in patients with homozygous familial hypercholesterolemia (HoFH). The policy specifies the approved HoFH dosing regimen as 15 mg/kg IV every 4 weeks, notes product vial strengths (for example, single‑dose vials such as 345 mg/2.3 mL), and requires prior authorization with documentation of diagnosis (genetic or clinical), current weight for dosing, and prior therapy/adherence information. Continued coverage requires evidence of clinical response (LDL‑C improvement) and adherence to concomitant therapies, with typical approval intervals and coding considerations updated to reflect current FDA labeling and coding guidance.

Definitions

HoFH definition

DefinitionHomozygous familial hypercholesterolemia (HoFH): a diagnosis defined by either pathogenic biallelic mutations (e.g., LDLR, PCSK9, APOB, LDLRAP1) or clinical criteria such as very elevated LDL-C and early xanthomas

Genetic evidenceHoFH may be established by mutations in LDLR, PCSK9, APOB, or LDLRAP1 genes

Clinical criteria examplesClinical HoFH criteria include untreated LDL-C ≥400 mg/dL, treated LDL-C ≥300 mg/dL, tendon or cutaneous xanthomas by age 10, or family history with elevated parental LDL-C

LDL-C definition

TermLDL-C = low density lipoprotein cholesterol

Context of useLDL-C laboratory results are required (recent labs referenced within 60 days for some initial criteria and within 3 months for continuation)

Policy Summary

PayerHealth Net

PolicyEvinacumab-dgnb (Evkeeza) coverage for HoFH

Policy CodePolicy N/A

Change TypeNo material change

Effective DateFeb 11, 2021

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation (weight in kg, recent LDL-C, genetic or clinical evidence of HoFH, prior therapies or intolerance) to support approval.

SourceLink

On This Page

Weight documentation and dosing limits: Current body weight in kilograms is documented and the requested dose does not exceed 15 mg/kg every 4 weeks.<= 15 mg/kg q4w

If request is for dose increase, new dose must not exceed the limit; document weight for pediatric dosing as applicable

Dose-change condition

If request is for a dose increase, the new dose must not exceed 15 mg/kg every 4 weeks

Age and LDL thresholds — pediatric and age-specific references

FDA pediatric age indication (policy updates)FDA pediatric extension referenced down to age ≥1 year in later updates; prior revisions noted age ≥12 years and other intermediate steps

Pediatric LDL leniencyA more lenient pediatric LDL-C requirement of 130 mg/dL was added in prior reviews for pediatric patients

Adult-specific LDL requirementsPolicy modifications clarified statin/ezetimibe requirements apply only to age ≥18 years in several revisions

Initial therapy: member must be on a statin at maximally tolerated dose unless statin therapy is contraindicated (see Appendices).
Documented statin intolerance requires evidence such as SAMS with re-challenge and titration attempts.
If age ≥18, member must have been adherent to ezetimibe concomitantly with a statin for at least 4 months when required.

Denial Risk

Contraindications and Hepatic Impairment

Providers must review contraindications and serious hepatic impairment considerations before prescribing. Requests should include documentation addressing contraindications (e.g., immune‑mediated hypersensitivity to HMG‑CoA reductase inhibitors, acute liver injury, rhabdomyolysis) and ezetimibe-related moderate to severe hepatic impairment (Child‑Pugh B or C) where applicable.

Statin contraindications: immune‑mediated hypersensitivity to the HMG‑CoA reductase inhibitor class (allergic reaction with at least two different statins), laboratory‑confirmed acute liver injury, or rhabdomyolysis related to statin therapy.
Ezetimibe contraindication: moderate or severe hepatic impairment (Child‑Pugh B or C).
Provide clinical documentation if statin or ezetimibe is not used due to contraindication or serious hepatic impairment.

Denial Risk

Non‑FDA Indications

Non‑FDA approved indications are not authorized under this policy unless sufficient documentation per the plan's off‑label use policies is provided.

Off‑label use must be supported by CP.CPA.09 (commercial) or CP.PMN.53 (Medicaid) off‑label use policy documentation, or evidence of coverage documents.
Requests lacking appropriate off‑label justification may be denied.

Note

Policy Revisions and Operational Notes

Policy revisions: documentation and program requirements have been updated over time — providers must follow the current FDA‑aligned labeling and policy requirements (including pediatric age and weight documentation, redirection guidance to alternative PCSK9 agents where applicable, and updated dosing/HCPCS coding).

Recent updates include revised pediatric age limits, requirement to document body weight, and HCPCS updates (e.g., J1305 for evinacumab).
Policy includes redirection guidance to alternative PCSK9 therapies (Praluent/Repatha) per clinical guidance and formulary preferencing; specific redirection rules may vary by age and product labeling.
Providers should reference the most current policy/version when submitting prior authorization requests.

Thresholds referencedMultiple LDL-C thresholds are used in criteria (e.g., ≥400 mg/dL untreated, ≥300 mg/dL treated, 130 mg/dL pediatric allowance)

SAMS — Statin-associated muscle symptoms

AcronymSAMS = statin-associated muscle symptoms

Clinical descriptionIncludes muscle pain, aching (myalgia), cramps, and weakness often bilateral and involving major muscle groups

Rechallenge relevanceReappearance of symptoms with statin rechallenge and disappearance with cessation provides best evidence of SAMS

LDLRAP1 (ARH) — gene definition

Gene nameLDLRAP1 = low density lipoprotein receptor adaptor protein 1

Alternate nameAlso known as autosomal recessive hypercholesterolemia (ARH) adaptor protein 1 gene

Role in HoFH assessmentMutations in LDLRAP1 are listed among genetic causes used to define HoFH in the policy

SAMS — expanded details

Core definitionStatin-associated muscle symptoms (SAMS): muscle pain, aching (myalgia), cramps, and weakness often bilateral and involving major muscle groups

Typical timing and courseSymptoms often appear early after starting statin therapy or after dose increase and usually resolve within weeks after stopping, though may take months to fully resolve

Persistence and evaluationPersistence of symptoms >2 months after cessation should prompt evaluation for other causes; documented disappearance with cessation and recurrence with rechallenge supports diagnosis