CurrentViva HealthPolicy IC-0708

Elfabrio (pegunigalsidase alfa-iwxj) IV for Fabry disease

Outlines prior authorization, initial and renewal clinical criteria, dosing, HCPCS/NDC coding, authorization lengths, and covered diagnosis for Elfabrio (pegunigalsidase alfa-iwxj) administered intravenously for treatment of Fabry disease.

Policy Summary

PayerViva Health

PolicyElfabrio (pegunigalsidase alfa-iwxj) IV for Fabry disease

Policy CodePolicy IC-0708

Change TypeClarified (no material change)

Effective DateJun 1, 2023

Next Review Date

Key ActionPrior authorization required; initial authorization valid for 12 months and may be renewed every 12 months; document biochemical or genetic confirmation and baseline biomarker or clinical findings.

POLICY UPDATE CHANGES

Document lists review dates 06/2023, 02/2024, 04/2025, 04/2026 and retains prior authorization length and criteria.

12 monthsInitial Authorization Length

115 mg q14dMax Dose per Interval

1Covered ICD-10 Indications

Coverage Summary

Covered with criteria for IV Elfabrio (pegunigalsidase alfa-iwxj) for Fabry disease; prior authorization required with initial authorization valid for 12 months (365 days) and may be renewed every 12 months (365 days). Scope includes prior authorization, initial and renewal clinical criteria, dosing, HCPCS/NDC coding, authorization lengths, and covered diagnosis for Elfabrio administered intravenously. Effective date: 2023-06-01; Last review: 2026-04-01.

Initial Approval Criteria

Prior authorization validity is provided when ALL of the following are met:

ALL of the following

ALL of the following
- ALL of the following
  - Member is at least 18 years of age
  - Must not be used in combination with migalastat or agalsidase beta
  - Fabry Disease (alpha-galactosidase A deficiency)
    ONE of
    Deficiency in α-galactosidase A (α-Gal A) activity in plasma, isolated leukocytes, and/or cultured cells (males only)
    Detection of pathogenic (or likely pathogenic) variants in the GLA gene by molecular genetic testing
    ONE of
    Tissue globotriaosylceramide (Gb3/GL-3) inclusions
    Plasma or urinary globotriaosylceramide (Gb3/GL-3) or globotriaosylsphingosine (lyso-Gb3)
    Clinical signs and/or symptoms of disease (e.g., dermatologic, gastrointestinal, pulmonary, vascular, renal, cardiac, neurologic manifestations)

Renewal / Continuation Criteria

Renewal Criteria

Prior authorization may be renewed every 12 months when ALL of the following are met:

ALL of the following

Member continues to meet the universal and other indication-specific relevant criteria identified in Initial Approval (section III), including concomitant therapy requirements (not including prerequisite therapy), performance status, etc.
Absence of unacceptable toxicity from the drug (examples: anaphylaxis, severe hypersensitivity reactions, severe infusion-associated reactions, glomerulonephritis)
Disease response (ONE of)
- Disease response defined by a reduction or stabilization in one or more of the following compared to pre-treatment baseline: Tissue Gb3/GL-3 inclusions; Plasma or urinary Gb3/GL-3 or lysoGb3
- Disease response defined by an improvement or stabilization of clinical signs and/or symptoms (e.g., dermatologic, gastrointestinal, pulmonary, vascular, renal, cardiac, neurologic manifestations)

Applicable Codes

HCPCSHCPCSCovered

J2508

Injection, pegunigalsidase alfa-iwxj, 1 mg; 1 billable unit = 1 mg

NDCNDC

10122-0165-xx	Elfabrio 5 mg/2.5 mL single-dose vial for injection
10122-0160-xx	Elfabrio 20 mg/10 mL single-dose vial for injection

Covered Diagnosis CodesICD-10Covered

E75.21

Fabry (-Anderson) disease

Billing unit: 1 billable unit = 1 mg (HCPCS J2508). Billing limit: maximum billed amount equivalent to 115 billable units (115 mg) every 14 days.

Provider Actions & Billing

Prior Authorization

Prior authorization required

Prior authorization required; initial authorization valid for 12 months (365 days) and may be renewed every 12 months thereafter.

Documentation Required

Diagnostic confirmation and baseline documentation

Provider must document biochemical or genetic confirmation of Fabry disease and a baseline value for at least one of: tissue Gb3 inclusions, plasma/urinary Gb3 or lyso-Gb3, or clinical signs/symptoms.

Documentation Required

Concomitant therapy restriction

Do not use in combination with migalastat or agalsidase beta; documentation of concomitant therapies should verify absence of these combinations.

Documentation Required

Renewal documentation of response and toxicity

For renewal, provider must document absence of unacceptable toxicity and disease response defined as reduction/stabilization in biomarkers (tissue Gb3, plasma/urinary Gb3 or lysoGb3) or improvement/stabilization of clinical signs/symptoms.

Billing Rule

HCPCS unit billing

Report J2508 with 1 billable unit = 1 mg; maximum billed amount equivalent to 115 billable units (115 mg) every 14 days.

J2508

Background

Background: Elfabrio is a PEGylated enzyme replacement therapy indicated for Fabry disease (alpha-galactosidase A deficiency). Dosing: 1 mg/kg IV every 2 weeks. Clinical confirmation requirements include biochemical or genetic confirmation of Fabry disease and a baseline value for at least one of tissue Gb3 inclusions, plasma/urinary Gb3 or lyso-Gb3, or clinical signs/symptoms. Baseline measures and renewal require documentation of absence of unacceptable toxicity and demonstration of disease response defined by reduction/stabilization in biomarkers (tissue Gb3, plasma/urinary Gb3 or lysoGb3) or improvement/stabilization of clinical signs/symptoms.

Policy facts

Policy numberIC-0708

SubjectElfabrio (pegunigalsidase alfa-iwxj) IV for Fabry disease

StatusCURRENT

Effective date2023-06-01

Last review date2026-04-01

Revision History

06/2023review

Document lists review dates and retains prior authorization length and criteria (initial authorization 12 months; renewal every 12 months).

02/2024review

Document lists review dates and retains prior authorization length and criteria (initial authorization 12 months; renewal every 12 months).

04/2025review

Document lists review dates and retains prior authorization length and criteria (initial authorization 12 months; renewal every 12 months).

04/01/2026reviewLatest

Latest review dated 04/01/2026; no specific material clinical changes indicated. Document retains prior authorization length and clinical criteria.

Policy Summary

PayerViva Health

PolicyElfabrio (pegunigalsidase alfa-iwxj) IV for Fabry disease

Policy CodePolicy IC-0708

Change TypeClarified (no material change)

Effective DateJun 1, 2023

Next Review Date

On This Page

OpenPayer

Elfabrio (pegunigalsidase alfa-iwxj) IV for Fabry disease

Coverage Summary

Initial Approval Criteria

Renewal / Continuation Criteria

Concomitant Therapy Exclusions

Applicable Codes

Provider Actions & Billing

Clinical Evidence & References

Background

Revision History