ModifiedCentenePolicy HIMPA.166

Evinacumab-dgnb (Evkeeza) for homozygous familial hypercholesterolemia

Coverage policy for Evkeeza (evinacumab-dgnb) as adjunct LDL-C lowering therapy for patients (including pediatrics >=5 years) with homozygous familial hypercholesterolemia (HoFH); defines initial and continued therapy criteria, dosing limits, and documentation requirements for Centene-affiliated plans (excluding CA Commercial Exchange per note).

Policy Summary

PayerCentene

PolicyEvinacumab-dgnb (Evkeeza) for homozygous familial hypercholesterolemia

Policy CodePolicy HIMPA.166

Change TypeCriteria and threshold updates (LDL thresholds, pediatric age, step therapy edits)

Effective Date03.01.23

Next Review Date

Key ActionSubmit prior authorization with documentation of weight, recent labs, prior therapy trials, and ensure dose does not exceed 15 mg/kg IV every 4 weeks.

SourceLink

POLICY UPDATE CHANGES

Lowered minimum LDL requirement to 55 mg/dL for members with ASCVD at very high risk and added corresponding Appendix H.

Added requirement for 8-week trial duration for redirection to Repatha and added Leqvio to list of potential co-administered drugs.

Updated pediatric FDA-approved age for HoFH from ≥12 years to ≥2–5 years (pediatric extension).

Lowered untreated LDL requirement to 400 mg/dL and revised familial hypercholesterolemia evidence to at least one parent; modified redirection to Repatha to apply only to age ≥10 years for HoFH.

Updated HCPCS codes with drug-specific code(s).

5 yrsminimum FDA‑approved pediatric age

15 mg/kgmaximum dose

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initial authorization

12 morenewal authorization

55 mg/dLvery high risk LDL threshold

Coverage Criteria for Evkeeza (evinacumab-dgnb)

Initial Therapy (HoFH)

Covered when ALL of the following are met for Initial Approval (HoFH):

HoFH diagnosis: Diagnosis of homozygous familial hypercholesterolemia (HoFH) defined by one of the following: a pathogenic genetic mutation consistent with HoFH (e.g., LDLR, PCSK9, apoB, LDLRAP1) OR untreated LDL-C ≥ 400 mg/dL plus one of the specified clinical features OR treated LDL-C ≥ 300 mg/dL; presence of tendinous or cutaneous xanthoma by age 10 years or evidence of familial hypercholesterolemia in a parent supports diagnosis.see document

supports diagnostic definition

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

required

Age ≥ 5 years (pediatric) or adult criteria as specified (age ≥18 years where noted).>=5 years

FDA‑approved age

LDL-C entry thresholds (recent within 60 days): For members age ≥5 and <18 years: recent LDL-C within the last 60 days as specified; for adults (≥18 years) recent LDL-C within the last 60 days meeting one of: LDL-C ≥ 270 mg/dL OR LDL-C ≥ 255 mg/dL if member has ASCVD and is at very high risk OR other adult thresholds as listed (treated LDL-C ≥300 mg/dL or untreated ≥400 mg/dL for diagnostic criteria).see document (e.g., ≥270 mg/dL; 255 mg/dL for very high risk ASCVD)

laboratory entry criteria

Statin and ezetimibe background therapy (adults ≥18 years): For adults ≥18 years receiving Evkeeza: Evkeeza must be prescribed in conjunction with a statin at maximally tolerated dose and the member must have been adherent to the maximally tolerated statin for at least the last 4 months; concomitant ezetimibe adherence for ≥4 months is required unless contraindicated or intolerant (see Appendix E/F).4 months adherence

exceptions for contraindication or intolerance documented in appendices

Statin intolerance pathway (adults not on statin therapy): Documentation of statin intolerance per Appendix G/E: trials of at least two statins (one hydrophilic recommended) or clinical history consistent with statin-associated muscle symptoms (SAMS) with appropriate rechallenge documentation, or documented contraindication to statin therapy.

see Appendix E and Appendix G for SAMS and contraindications

Prior biologic/step therapy: For age ≥10 years, failure of an 8-week trial of evolocumab (Repatha) is required unless contraindicated or if member has ≤2% LDLR activity; if coadministration with agents such as Praluent, Juxtapid, Leqvio, or Repatha is requested, prior therapy with those agents must have been tried for at least 3 consecutive months with inadequate response as specified.8 weeks for Repatha; 3 months for other agents

step therapy requirements and redirection policies

Dosing and documentation requirements: Dose must not exceed 15 mg/kg administered IV every 4 weeks; documentation of the member's current weight in kg is required; initial approval duration is 6 months.15 mg/kg q4w

dose cap and initial authorization length

Continued Therapy (HoFH)

Covered when ALL of the following are met for Continued Therapy:

Prior approval/continuity: Member is currently receiving Evkeeza via the Centene benefit or previously met initial approval criteria; continuity of care provisions apply per state/product addenda.

continuation provision

Efficacy response: Member is responding positively to therapy as evidenced by laboratory results within the last 3 months showing an LDL‑C reduction since initiation of Evkeeza.documented LDL‑C reduction within 3 months

required for renewal

Statin adherence for statin‑tolerant adults: If statin tolerant, documentation of adherence to a statin at the maximally tolerated dose is required for continued coverage.

applies to adults

Not Medically Necessary / Not Authorized Uses

Not authorized:

Non‑FDA indications: Coverage is not authorized for non‑FDA approved indications that are not addressed in this policy unless there is sufficient documentation of efficacy and safety consistent with the off‑label use policy (see HIM.PA.154) or evidence of coverage documents.

refer to off‑label policy HIM.PA.154

Appendix-based clinical criteria

Clinical definitions and qualifying conditions included in appendices relevant to coverage decisions:

Very High ASCVD Risk Definition: Very high risk for future ASCVD events is defined as either a history of multiple major ASCVD events OR one major ASCVD event plus multiple high‑risk conditions.

Major ASCVD events and high‑risk conditions are listed in Appendix H (examples: recent ACS within 12 months; prior MI; PAD; ischemic stroke; HeFH; age ≥65 years; diabetes; CKD; persistently elevated LDL‑C despite therapy).

HoFH Indication and Dosing: Evkeeza dosing for HoFH: 15 mg/kg IV every 4 weeks; product vial strengths and administration details are provided in the dosing section.15 mg/kg q4w

See Dosage and Administration

Low Intensity Statin Examples: Examples of low‑intensity statin regimens used to classify prior therapy: simvastatin 10 mg; pravastatin 10–20 mg; fluvastatin 20–40 mg; lovastatin 20 mg (expected LDL‑C reduction on average <30%).

Requests for uses of evinacumab‑dgnb (Evkeeza) that are for non‑FDA approved indications not specifically addressed in this policy are excluded from coverage unless the provider supplies sufficient documentation of efficacy and safety in accordance with the plan’s off‑label use policy (see HIM.PA.154) or applicable evidence‑of‑coverage documents.

When assessing required trials of background lipid‑lowering therapy, prescribers should document any contraindications or intolerance to statins or ezetimibe. Contraindications for statins cited in the policy include decompensated liver disease (signs such as jaundice, ascites, variceal bleeding, encephalopathy), laboratory‑confirmed acute liver injury or rhabdomyolysis attributable to statin therapy, pregnancy/actively trying to conceive/nursing, and immune‑mediated hypersensitivity to two different statins. Ezetimibe contraindications include moderate to severe hepatic impairment (Child‑Pugh B or C) and hypersensitivity to ezetimibe. These contraindications may be used to justify exceptions to required trials of statin or ezetimibe per the policy appendices.

Use of evinacumab‑dgnb for non‑FDA approved indications without adequate supporting clinical documentation as required by the plan’s off‑label use policy will be considered not authorized (not medically necessary) under this policy.

Coding information included in this policy is provided for reference only. Inclusion or exclusion of codes does not guarantee coverage; providers must use current professional coding guidance when submitting claims and confirm coverage determinations per the member’s benefit plan.

Coding and Dose Thresholds

HCPCS CodesHCPCS

J1305

Injection, evinacumab-dgnb, 5 mg

LDL-C thresholds

Untreated LDL-C thresholdUntreated LDL-C ≥ 400 mg/dL (used to support HoFH diagnosis)

Treated LDL-C thresholdTreated LDL-C ≥ 300 mg/dL (alternative diagnostic criterion)

Other referenced LDL examplesAdditional referenced LDL‑C values used in criteria: ≥270 mg/dL; ≥255 mg/dL for ASCVD with very high risk context

Maximum dose

Maximum single dosing limitDose must not exceed 15 mg/kg every 4 weeks

Documentation requirement for dosingProvider must document member's current weight in kg to calculate dose

Provider Actions, Prior Authorization & Documentation

Prior Authorization

Prior Authorization Required & Dosing Limits

Prior authorization is required for Evkeeza; initial approval length is 6 months (12 months for ongoing HoFH therapy). Dose must not exceed 15 mg/kg every 4 weeks and requests for dose increases must not exceed this maximum. Providers must include documentation of the member’s current weight in kg to support dosing.

Initial approval duration: 6 months (continuation/HoFH: 12 months when criteria met)
Maximum dose: 15 mg/kg IV every 4 weeks
Provide current weight in kg with all requests

Documentation Required

Prior Authorization Expectations & Required Documentation

Prior authorization criteria have been updated over time (LDL thresholds, age limits, trial and redirection requirements). When submitting a request, include contemporaneous clinical documentation (office notes, lab results, prior medication trials) showing the member meets the current policy criteria.

Definitions and Clinical Terms

Homozygous familial hypercholesterolemia (HoFH)

Genetic basisHoFH diagnosis may be supported by pathogenic mutations in LDLR, PCSK9, apoB, or LDLRAP1 genes

Laboratory phenotypeVery high untreated LDL‑C (e.g., ≥400 mg/dL) or treated LDL‑C ≥300 mg/dL used as diagnostic support

Clinical phenotypePhysical findings such as tendinous or cutaneous xanthomas (including onset by age 10) support HoFH diagnosis

Low Intensity Statin Therapy

DefinitionLow‑intensity statin therapy: daily dosing expected to lower LDL‑C on average by <30%

ExamplesSimvastatin 10 mg; Pravastatin 10–20 mg; Fluvastatin 20–40 mg; Lovastatin 20 mg

Background

Evinacumab‑dgnb (Evkeeza) is a monoclonal antibody that targets angiopoietin‑like protein 3 (ANGPTL3), relieving ANGPTL3‑mediated inhibition of lipoprotein lipase and thereby reducing circulating LDL‑cholesterol. It is indicated as adjunct LDL‑C lowering therapy for patients with homozygous familial hypercholesterolemia (HoFH). The approved HoFH dosing regimen is 15 mg/kg administered intravenously every 4 weeks. Clinical benefit on cardiovascular morbidity and mortality has not been established, and safety and effectiveness for other causes of hypercholesterolemia (for example, heterozygous familial hypercholesterolemia) are not established in the labeling.

Policy Summary

PayerCentene

PolicyEvinacumab-dgnb (Evkeeza) for homozygous familial hypercholesterolemia

Policy CodePolicy HIMPA.166

Change TypeCriteria and threshold updates (LDL thresholds, pediatric age, step therapy edits)

Effective Date03.01.23

Next Review Date

Key ActionSubmit prior authorization with documentation of weight, recent labs, prior therapy trials, and ensure dose does not exceed 15 mg/kg IV every 4 weeks.

SourceLink

On This Page

Weight and dosing for continuation: Documentation of current weight in kg is required; if a dose increase is requested the new dose must not exceed 15 mg/kg every 4 weeks.15 mg/kg q4w

dose cap for renewal

LDL reduction <30% on average

Used to classify statin intensity when assessing prior therapy/trials.

Statin and Ezetimibe Contraindications: Statin contraindications include decompensated liver disease, laboratory‑confirmed acute liver injury or rhabdomyolysis from statin treatment, pregnancy/actively trying to conceive/nursing, and immune‑mediated hypersensitivity to the HMG‑CoA reductase inhibitor class; ezetimibe contraindications include moderate to severe hepatic impairment and hypersensitivity reactions.

Appendix E lists contraindications; FDA pregnancy language updated historically but appendix retained for clinical guidance.

Statin Risk Factors: Statin risk factors used when assessing tolerance include: multiple or serious comorbidities (impaired renal or hepatic function), unexplained ALT elevations ≥3× ULN or active liver disease, age >75 years, history of hemorrhagic stroke, concomitant interacting drugs, and Asian ancestry.

Appendix F provides details for risk assessment.

SAMS (Statin‑Associated Muscle Symptoms) guidance: Clinical features of SAMS (myalgia, cramps, weakness, typical distribution and timing, and approach to rechallenge) are described in Appendix G and inform documentation of statin intolerance.

Appendix G describes SAMS diagnostic considerations and rechallenge evidence.

Approval duration linked to dosingInitial approval duration is 6 months; continued therapy reviews include dose cap for increases

LDL thresholds (policy updates)

Lowered LDL for very high risk ASCVDMinimum LDL requirement lowered to 55 mg/dL for members with ASCVD at very high risk (per 2022 ACC pathway)

Lowered untreated LDL requirement (1Q2025)Untreated LDL requirement lowered to 400 mg/dL (1Q 2025 annual review)

Policy history of LDL threshold changesPolicy updates and reviews document stepwise lowering of LDL thresholds and corresponding Appendix H additions

Include office chart notes and laboratory results
Document previous medication trials, contraindications, or intolerance
Off‑label indications not addressed in this policy require sufficient supporting evidence per off‑label policy (HIM.PA.154)

Step Therapy

Prior Therapy Trial for Coadministration

For requests involving coadministration with other lipid-lowering agents (Juxtapid, Leqvio, Praluent, or Repatha), the member must have tried the prior therapy for at least 3 consecutive months and demonstrated inadequate response, defined as failure to achieve LDL-C <250 mg/dL or failure to achieve a ≥20% reduction in LDL-C from baseline.

Required trial duration for coadministered agents: ≥3 consecutive months
Inadequate response = LDL-C ≥250 mg/dL OR <20% reduction from baseline

Step Therapy

Redirection / Step to PCSK9 Inhibitor (Repatha)

Per policy updates, redirection (step) to a PCSK9 inhibitor (Repatha) is required for certain ages. For members aged ≥10 years, a trial of Repatha for 8 weeks is required prior to redirection unless Repatha is contraindicated, causes clinically significant adverse effects, or the member has ≤2% LDLR activity. Operationally, document the 8‑week trial and its outcome when requesting redirection.

Repatha trial required for ages ≥10 years: 8 weeks
Exceptions: contraindication, clinically significant adverse effects, or ≤2% LDLR activity
Document trial duration and clinical response

Use in policyClassified to assess prior therapy trials and intolerance pathways when evaluating eligibility

Very High Risk for ASCVD

Core definitionVery high risk for ASCVD: either multiple major ASCVD events OR one major ASCVD event plus multiple high‑risk conditions

Major ASCVD events (examples)Recent acute coronary syndrome (within 12 months); prior myocardial infarction; ischemic stroke; symptomatic peripheral artery disease

High‑risk conditions (examples)HeFH, age ≥65 years, prior coronary revascularization, diabetes, hypertension, chronic kidney disease, persistent LDL‑C ≥100 mg/dL despite therapy, smoking, heart failure

HoFH dosing

HoFH dosing regimenEvinacumab 15 mg/kg administered IV every 4 weeks for homozygous familial hypercholesterolemia

Dose cap for changesIf a dose increase is requested for continued therapy, the new dose must not exceed 15 mg/kg every 4 weeks

Supporting documentationProduct vial strengths and dosing availability listed in dosing and product availability sections to support administration and billing