CurrentCentenePolicy CP PHAR.512

Pegunigalsidase Alfa-iwxj (Elfabrio)

Defines medical necessity criteria, initial and continued approval conditions, dosing limits, exclusions (concomitant therapy), required documentation, approval durations, and coding implications for Elfabrio across Commercial, HIM, and Medicaid lines of business.

Policy Summary

PayerCentene

PolicyPegunigalsidase Alfa-iwxj (Elfabrio)

Policy CodePolicy CP PHAR.512

Change TypeCoding, Criteria Clarification, Dosing Revision

Effective Date05.23

Next Review Date

Key ActionProvider must submit documentation (office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.

POLICY UPDATE CHANGES

Added concomitant use exclusion to the Continued Therapy section to mirror Initial Approval Criteria.

Added HCPCS code J2508 for pegunigalsidase alfa-iwxj.

Removed prior requirement to document three specific Fabry symptoms for initial coverage; aligned criteria with product labeling.

1FDA-approved indication

18+Minimum age (years)

1 mg/kg q2wMaximum dosing

J2508Key HCPCS code

Coverage stance: Elfabrio (pegunigalsidase alfa-iwxj) is covered with criteria for the treatment of adults with confirmed Fabry disease, aligned to FDA labeling. Coverage applies across Commercial, HIM, and Medicaid lines of business. Initial approvals are granted for 6 months and continued therapy approvals for 12 months following medical necessity review.

Eligibility and prescribing limits: patients must be >= 18 years of age, have a confirmed diagnosis of Fabry disease per enzyme assay or DNA testing, and the medication must be prescribed by or in consultation with an appropriate specialist (clinical geneticist, cardiologist, nephrologist, neurologist, lysosomal disease specialist, or Fabry disease specialist). Dosing must not exceed 1 mg/kg every 2 weeks, and documentation of the member's current weight (kg) is required for dose calculation.

Concomitant therapy and administrative requirements: Elfabrio is not authorized concurrently with other Fabry therapies such as Fabrazyme or Galafold; requests may be denied if concurrent use is documented. Providers must submit supporting documentation (office notes, labs, or other clinical information) to demonstrate criteria are met and follow prior authorization procedures.

Initial Therapy Criteria

Initial Approval Criteria

Elfabrio is medically necessary when ALL of the following are met:

ALL of the following

ONE of
- Enzyme assay demonstrating a deficiency of alpha-galactosidase activity
- DNA testing
Prescribed by or in consultation with a clinical geneticist, cardiologist, nephrologist, neurologist, lysosomal disease specialist, or Fabry disease specialist
Patient age is >= 18 years>= 18 years
Documentation of member's current weight (in kg)
Elfabrio is not prescribed concurrently with Fabrazyme or Galafold
Dose does not exceed 1 mg/kg every 2 weeks1 mg/kg every 2 weeks
Maximum dosing limit for initial approval

Documentation Required

Supportive documentation required

Provider must submit supportive documentation such as office chart notes, lab results, or other clinical information demonstrating that the member meets all approval criteria.

Office chart notes
Laboratory results
Other clinical information

Continuation Therapy Criteria

Continued Therapy Criteria

Continue Elfabrio when ALL of the following are met:

ALL of the following

Member is currently receiving medication via Centene benefit or member has previously met initial approval criteria
Member is responding positively to therapy as evidenced by improvement in the individual member's Fabry disease manifestation profile
Documentation of member's current weight (in kg)
Elfabrio is not prescribed concurrently with Fabrazyme or Galafold
If request is for a dose increase, new dose does not exceed 1 mg/kg every 2 weeks1 mg/kg every 2 weeks

Documentation Required

Supportive documentation required — continued therapy

Submit office notes, lab results, or other clinical information to demonstrate continued benefit and that criteria are met, including documentation of current weight (kg) for dosing.

Office notes
Lab results
Other clinical information
Document current weight (kg)

Unproven / Exclusions

Diagnoses/Indications Not Authorized

Coverage is NOT authorized for:

ALL of the following

Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off-label use policies CP.CPA.09 (commercial), HIMPA.154 (HIX), and CP.PMN.53 (Medicaid), or evidence of coverage documents

Denial Risk

Concomitant therapy exclusion

Request may be denied if Elfabrio is prescribed concurrently with Fabrazyme or Galafold. Ensure therapies are not used concomitantly when submitting authorization requests.

Concurrent use with Fabrazyme or Galafold may result in denial

Applicable Codes

HCPCS (drug-specific)HCPCS

J2508

Injection, pegunigalsidase alfa-iwxj, 1 mg

Billing Rule

Dosing and weight documentation (billing rule)

Document the member's current weight (kg) for dose calculation and ensure billed dosing does not exceed the policy maximum of 1 mg/kg every 2 weeks when submitting claims.

J2508

Provider Actions

Prior Authorization

Prior authorization required

Requests are subject to medical necessity review and require prior authorization. Initial approval duration is 6 months; continued therapy approvals are 12 months.

Initial approval: 6 months
Continued therapy approval: 12 months

Documentation Required

Supportive documentation required

Provider must submit documentation (office chart notes, lab results, or other clinical information) to support that the member has met all approval criteria.

Office chart notes
Lab results
Other clinical information

Billing Rule

Dosing & weight documentation (billing rule)

Document member's current weight (kg) for dose calculation and ensure billed dose does not exceed 1 mg/kg every 2 weeks. Include the HCPCS code J2508 on claims for pegunigalsidase alfa-iwxj.

Weight (kg) must be documented
Maximum dosing: 1 mg/kg every 2 weeks
J2508

Denial Risk

Concomitant therapy exclusion

Request may be denied if Elfabrio is prescribed concurrently with Fabrazyme or Galafold; do not submit concurrent therapy requests for authorization.

Clinical Evidence

Evidence sources include the Elfabrio Prescribing Information (Chiesi Farmaceutici; 2024) and referenced clinical trials. Key cited studies are a Phase III open-label switch-over study (poster abstract) and a Phase 1/2 trial reporting sustained plasma concentrations and favorable pharmacodynamics over one year.

The policy reference list also cites additional background and consensus literature on Fabry disease management and includes the prescribing information as the primary regulatory source for indications, dosing, and safety.

Background & Definitions

Background: Pegunigalsidase alfa-iwxj (Elfabrio) is an enzyme replacement therapy (a hydrolytic lysosomal glycosphingolipid enzyme) indicated for the treatment of adults with confirmed Fabry disease and the policy aligns coverage with FDA labeling.

Clinical and administrative requirements: prescribing must be by or in consultation with a specialist (clinical geneticist, cardiologist, nephrologist, neurologist, lysosomal disease specialist, or Fabry disease specialist). The policy requires documentation of the member's current weight (kg) for dosing and specifies that Elfabrio must not be prescribed concurrently with Fabrazyme or Galafold. The approved dosing regimen is 1 mg/kg every 2 weeks with vial presentations noted in product availability.

Definition: Fabry disease is defined in the policy as "an X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A leading to glycosphingolipid accumulation."

Revision History

05.25.2025policy_updateLatest

Added concomitant use exclusion to the Continued Therapy section to mirror Initial Approval Criteria; added requirement for documentation of member's weight for dose calculation purposes.

08.23coding_update

Added HCPCS code J2508 for pegunigalsidase alfa-iwxj.

05.15.23criteria_revision

Removed prior requirement to document three specific Fabry symptoms for initial coverage; added Galafold to Fabrazyme as an excluded medication for concomitant coverage; removed earlier maximum dosing limit of 2 mg/kg every 4 weeks because product was not approved for that regimen.

clarification

Also added requirement for documentation of member's weight for dose calculation purposes.

Policy Summary

PayerCentene

PolicyPegunigalsidase Alfa-iwxj (Elfabrio)

Policy CodePolicy CP PHAR.512

Change TypeCoding, Criteria Clarification, Dosing Revision

Effective Date05.23

Next Review Date

Key ActionProvider must submit documentation (office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.

On This Page

OpenPayer

Pegunigalsidase Alfa-iwxj (Elfabrio)

Coverage Summary

Initial Therapy Criteria

Continuation Therapy Criteria

Unproven / Exclusions

Applicable Codes

Provider Actions

Clinical Evidence

Background & Definitions

Revision History