CurrentCignaPolicy IP0279

Pompe Disease - Enzyme Replacement Therapy - Nexviazyme

Defines Cigna coverage and prior authorization criteria, dosing, and coding for Nexviazyme (avalglucosidase alfa-ngpt) for treatment of late-onset Pompe disease in patients aged ≥1 year. Also states non-covered uses and prescribing specialist requirements.

Policy Summary

PayerCigna

PolicyPompe Disease - Enzyme Replacement Therapy - Nexviazyme

Policy CodePolicy IP0279

Change TypePolicy name updated; no criteria changes

Effective DateJul 15, 2025

Next Review Date

Key ActionPrior Authorization is required for Nexviazyme; approve when the patient meets the stated criteria and dosing for a 1-year duration.

POLICY UPDATE CHANGES

Policy Name changed from 'Avalglucosidase' to 'Pompe Disease - Enzyme Replacement Therapy - Nexviazyme.'

1Covered Indication (late-onset Pompe disease)

1 yearInitial approval duration

2Weight-based dosing regimens

Coverage Summary

Coverage stance: covered with criteria for Nexviazyme (avalglucosidase alfa-ngpt) for late-onset Pompe disease in patients aged ≥ 1 year. Policy Number: IP0279. Effective date: 7/15/2025. Last review / policy update: 7/15/2025 (annual revision noted 08/15/2024 and 07/15/2025). Scope: defines Cigna coverage and prior authorization criteria, dosing, and coding for Nexviazyme for treatment of late-onset Pompe disease in patients aged ≥1 year, with non-covered uses and prescribing specialist requirements.

FDA-Approved Indication (Approve for 1 year)

Approve for 1 year if the patient meets ALL of the following (A, B, C, and D):

ALL of the following

A: Patient is ≥ 1 year of age
B: Patient has late-onset acid alpha-glucosidase deficiency (late-onset Pompe disease)
C
- i: Laboratory test demonstrating deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue
- ii: Molecular genetic test demonstrating biallelic pathogenic or likely pathogenic acid alpha-glucosidase (GAA) gene variants
D: Medication is prescribed by or in consultation with a geneticist, neurologist, a metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders

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Dosing (Approve ONE)

Approve ONE of the following dosing regimens (A or B):

ANY of the following

A: Patient ≥ 30 kg: Dose is 20 mg/kg administered by intravenous infusion once every 2 weeks
B: Patient < 30 kg: Dose is 40 mg/kg administered by intravenous infusion once every 2 weeks

Provider Actions / Prior Authorization

Prior Authorization

Prior authorization required

Prior Authorization is required for benefit coverage of Nexviazyme. Approval is recommended when the patient meets the stated criteria and dosing and approvals are provided for a duration of 1 year.

J0219

Documentation Required

Diagnosis confirmation required

Require documentation of diagnosis by ONE of the following: laboratory demonstration of deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue OR molecular genetic testing demonstrating biallelic pathogenic or likely pathogenic GAA variants. Documentation must also include prescriber specialty or consultation information.

Documentation Required

Specialist prescriber requirement

Nexviazyme must be prescribed by or in consultation with a geneticist, neurologist, a metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders. Prescriber specialty or consultation documentation is required.

Billing Rule

Dose requests outside policy

Requests for doses outside the established dosing regimens documented in this policy will be considered on a case-by-case basis by a clinician (Medical Director or Pharmacist).

J0219

Clinical Evidence & References

Evidence: FDA prescribing information: Nexviazyme intravenous infusion PI, Genzyme; September 2023. This evidence supports the FDA‑approved indication for Nexviazyme in acid alpha‑glucosidase deficiency (Pompe disease).

Definitions / background: Late-onset Pompe disease — Pompe disease with onset after 12 months of age, presenting with progressive muscle weakness and possible respiratory insufficiency.

Background

Nexviazyme is a recombinant human acid alpha‑glucosidase enzyme indicated for late‑onset Pompe disease in patients aged ≥ 1 year. Pompe disease is a lysosomal storage disorder caused by deficiency of acid alpha‑glucosidase (GAA), leading to accumulation of glycogen primarily in muscle. The onset, progression, and severity are variable: infantile‑onset presents in the first months of life with hypotonia and cardiopulmonary failure if untreated, while late‑onset disease can present any time after 12 months with progressive muscle weakness that can progress to respiratory insufficiency. Diagnosis is by demonstrating decreased acid alpha‑glucosidase activity in blood, fibroblasts, or muscle tissue, or by genetic testing.

Policy Metadata

Policy NumberIP0279

Effective Date2025-07-15

Last Review2025-07-15

StatusCURRENT

Scope SummaryDefines Cigna coverage and prior authorization criteria, dosing, and coding for Nexviazyme (avalglucosidase alfa-ngpt) for treatment of late-onset Pompe disease in patients aged ≥1 year. Also states non-covered uses and prescribing specialist requirements.

Revision History

08/15/2024revised

Annual Revision: Policy Name changed from 'Avalglucosidase' to 'Pompe Disease - Enzyme Replacement Therapy - Nexviazyme.' No criteria changes.

07/15/2025revisedLatest

Annual Revision dated 07/15/2025: Policy name updated to 'Pompe Disease - Enzyme Replacement Therapy - Nexviazyme.' No criteria changes. Policy effective date 7/15/2025.

Policy Summary

PayerCigna

PolicyPompe Disease - Enzyme Replacement Therapy - Nexviazyme

Policy CodePolicy IP0279

Change TypePolicy name updated; no criteria changes

Effective DateJul 15, 2025

Next Review Date

Key ActionPrior Authorization is required for Nexviazyme; approve when the patient meets the stated criteria and dosing for a 1-year duration.

On This Page

OpenPayer

Pompe Disease - Enzyme Replacement Therapy - Nexviazyme

Coverage Summary

Provider Actions / Prior Authorization

Applicable Codes

Clinical Evidence & References

Background

Revision History