CurrentCignaPolicy IP0406

Fabrazyme (agalsidase beta) Coverage

Defines prior authorization, medical necessity criteria, dosing limits, and exclusions for Fabrazyme (agalsidase beta) for treatment of Fabry disease for Cigna-administered health plans.

Policy Summary

PayerCigna

PolicyFabrazyme (agalsidase beta) coverage

Policy CodePolicy IP0406

Change TypeTitle updatedosing limit addedreauthorization removedconcurrent therapy restriction added

Effective DateJul 15, 2025

Next Review Date

Key ActionObtain prior authorization and document specialist diagnosis confirmation; approve for 1 year when criteria and dosing limits (≤1 mg/kg IV every ≥2 weeks) are met.

SourceLink

POLICY UPDATE CHANGES

Added dosing for Fabry disease (each dose must not exceed 1 mg/kg IV no more frequently than once every 2 weeks).

Added Concurrent Use with Elfabrio (pegunigalsidase alfa) as a condition not covered.

Removed reauthorization criteria.

Updated coverage policy title from Agalsidase to Enzyme Replacement Therapy - Fabrazyme.

1 yearinitial approval duration for FDA-approved indication

1 mg/kgmaximum dose per administration

every 2 weeksminimum dosing interval

J0180

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Fabrazyme (agalsidase beta)

Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

FDA-Approved Indication: Fabry Disease: Approve for 1 year if the patient meets BOTH of the following (A and B): A) The diagnosis is established by ONE of the following: i. laboratory test demonstrating deficient alpha-galactosidase A activity in leukocytes or fibroblasts; OR ii. molecular genetic test demonstrating a pathogenic variant in the galactosidase alpha (GLA) gene; AND B) Fabrazyme is prescribed by or in consultation with a geneticist, endocrinologist, a metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders.Each dose must not exceed 1 mg/kg administered intravenously no more frequently than once every 2 weeks.

Prior authorization required; approve for 1 year when criteria are met.

Fabrazyme (agalsidase beta) is covered only for the treatment of Fabry disease. Use of Fabrazyme for any indication other than Fabry disease is considered not medically necessary and will not be covered. Concurrent administration of Fabrazyme with other specified therapies is addressed separately under the policy exclusions and is not permitted as described below.

Concurrent use of Fabrazyme with Galafold (migalastat) is considered not medically necessary. The policy notes that available data are limited — one small study evaluated a single dose of migalastat given with Fabrazyme and, although a transient increase in α‑Gal activity was observed, the long‑term safety and efficacy of concurrent use has not been established. Additionally, migalastat is not FDA‑approved for use in combination with Fabrazyme; therefore concurrent use is excluded from coverage.

Concurrent use of Fabrazyme with Elfabrio (pegunigalsidase alfa) is explicitly listed as not medically necessary and will not be covered. This exclusion was added in the policy revision history and applies to requests for simultaneous administration of these enzyme replacement therapies.

Coding and Dosing Limits

Considered Medically Necessary HCPCS CodesHCPCSCovered

J0180

Injection, agalsidase beta, 1 mg

inv-06: Dose limit — Each dose must not exceed 1 mg/kg IV no more frequently than once every 2 weeks

Dose per administrationEach dose must not exceed 1 mg/kg administered intravenously

Maximum dosing frequencyNo more frequently than once every 2 weeks

Administration routeIntravenous (IV)

Approval duration tied to dosingApproved for up to 1 year when criteria and dosing are met

inv-16: Fabrazyme (agalsidase beta) quantity limit — 1 mg/kg per dose

Quantity limit per dose1 mg/kg per dose

Provider Actions, Prior Authorization, and Restrictions

Prior Authorization

Prior Authorization Required

Prior Authorization is required for benefit coverage of Fabrazyme. Approvals are recommended for 1 year when the patient meets the clinical Criteria and Dosing in the policy. Extended approvals may be granted if the patient continues to meet criteria. Requests for doses outside the established dosing will be considered case-by-case by a clinician (e.g., Medical Director or Pharmacist). All approvals are provided for the duration noted. Use HCPCS code J0180 for billing (Injection, agalsidase beta, 1 mg).

Prior authorization required for Fabrazyme
Approve for up to 1 year when criteria are met; extensions allowed if criteria continue to be met
Case-by-case review for doses outside established dosing
Billing HCPCS: J0180 (Injection, agalsidase beta, 1 mg)

Documentation Required

Specialist Prescriber and Diagnostic Documentation

Initial Therapy Medical Necessity Criteria

Initial Therapy Criteria — 1 top-level node

Initial Approval Criteria: Diagnosis confirmed by deficient alpha-galactosidase A activity (laboratory) OR a pathogenic GLA gene variant, AND Fabrazyme is prescribed by or in consultation with a geneticist, endocrinologist, metabolic disorder sub-specialist, or physician who specializes in lysosomal storage disorders. Approve for 1 year when criteria are met.Each dose must not exceed 1 mg/kg IV; dosing interval no more frequently than once every 2 weeks.

Continuation / Extension Criteria

Continuation/Extension — criteria for extended approvals

Extended approvals

Continuation: Extended approvals allowed if the patient continues to meet the Criteria and Dosing specified in the policy. Requests for doses outside the established dosing will be considered case-by-case by a clinician.Approvals provided for the duration noted (e.g., 1 year).

Prior authorization remains required; prescription by or in consultation with an appropriate specialist is required.

Step-Therapy and Related Restrictions

Concurrent therapy	Policy status	Notes / Rationale
Galafold (migalastat)	Not Covered	One small study (n = 23) assessed a single dose used concurrently with Fabrazyme; long-term safety and efficacy of concurrent use has not been established. Galafold is not FDA‑approved for concurrent use with Fabrazyme.
Elfabrio (pegunigalsidase alfa)	Not Covered	Concurrent use with Elfabrio is listed as not medically necessary.

Site-of-Care and Administration

Note

Infusion center — prescribe by or in consultation with appropriate specialist

Because evaluation and monitoring require specialized skills, Fabrazyme should be prescribed by or in consultation with an appropriate specialist (as listed in the policy) when administered in an infusion center.

Specialist involvement required due to need for specialized evaluation, diagnosis, and monitoring of adverse events and long-term efficacy.
Site of care is an infusion center, but prescription must involve an appropriate specialist or consultation.

Background on Fabrazyme and Fabry Disease

Fabrazyme (agalsidase beta) is an enzyme replacement therapy indicated for Fabry disease, a rare X‑linked lysosomal storage disorder caused by deficient α‑galactosidase A activity leading to accumulation of globotriaosylceramide (GL‑3). Disease progression can involve the kidneys, heart, and nervous system and may result in proteinuria, reduced glomerular filtration rate, end‑stage renal disease, cardiomyopathy, arrhythmias, and stroke. Fabrazyme works by replacing the deficient enzyme to reduce GL‑3 deposition and help slow organ damage.

Definitions

inv-12: Fabry disease definition — rare X-linked lysosomal storage disorder due to deficient alpha-galactosidase A activity

DefinitionA rare X-linked lysosomal storage disorder due to deficient alpha-galactosidase A (α-Gal) activity resulting in accumulation of globotriaosylceramide (GL-3)

PathophysiologyGL-3 accumulation occurs across many cell types and leads to progressive multisystem disease affecting kidney, heart, and nervous system

Genetics and diagnosisDiagnosis can be confirmed by deficient α-Gal activity in leukocytes/fibroblasts or by identifying a pathogenic GLA gene variant

Clinical consequencesLong-term consequences include hypertrophic cardiomyopathy, arrhythmias, renal failure, stroke, progressive proteinuria, and decline in GFR potentially leading to end-stage renal disease