CurrentCentenePolicy CP PHAR.521

Clinical Policy: Avalglucosidase Alfa-ngpt (Nexviazyme)

Defines medical necessity, authorization, dosing, and continuation criteria for Nexviazyme in patients (≥1 year) with late-onset Pompe disease for Centene lines of business.

Policy Summary

PayerCentene

PolicyAvalglucosidase Alfa-ngpt (Nexviazyme) clinical policy

Policy CodePolicy CP PHAR.521

Change TypeNo material change

Effective DateAug 6, 2021

Next Review Date

Key ActionObtain prior authorization with documentation confirming diagnosis of late‑onset Pompe disease and supporting clinical tests.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥1 yrFDA‑approved minimum age

20 mg/kgDose ≥30 kg

40 mg/kgDose <30 kg

J0219HCPCS code

6 moInitial approval duration

Coverage Criteria

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inv-01: Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met:

Diagnostic confirmation: Diagnosis of late‑onset Pompe disease confirmed by one of the following: enzyme assay confirming low GAA activity; DNA testing; or increased lysosomal glycogen.

From initial criteria list

No concurrent enzyme therapies: Nexviazyme is not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda.

Concurrent therapy exclusion

Weight‑based dosing: For members weighing ≥30 kg: 20 mg/kg every 2 weeks; for members weighing <30 kg: 40 mg/kg every 2 weeks.weight 30 kg

Dose limits provided

inv-02: Continuation Therapy — Covered for continuation when ALL of the following are met

Covered for continuation when ALL of the following are met:

Current therapy or prior criteria met: Member is currently receiving Nexviazyme via Centene benefit or has previously met initial approval criteria; continuity of care rules may apply.

Continuation eligibility

Clinical response: Member is responding positively to therapy as evidenced by improvement or maintenance of Pompe disease manifestations (examples include improved or maintained forced vital capacity [FVC] or 6 minute walk test [6MWT]).

Clinical benefit required; see Appendix D for examples

Dose limits for continuation: If request is for a dose increase, the new dose must not exceed weight‑based maximums: ≥30 kg = 20 mg/kg every 2 weeks; <30 kg = 40 mg/kg every 2 weeks.

Dose cap

No concurrent enzyme therapies: Nexviazyme is not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda.

Concurrent therapy exclusion

Coverage is not authorized for indications that are not FDA‑approved unless the provider submits sufficient supporting documentation demonstrating efficacy and safety per the referenced off‑label use policies. Acceptable documentation includes evidence consistent with the plan’s off‑label policies: CP.CPA.09 (commercial), HIM.PA.154 (health insurance marketplace), or CP.PMN.53 (Medicaid), or other documented evidence of coverage that supports the off‑label use.

The policy was updated in the 2Q 2024 annual review to add an explicit exclusion prohibiting concomitant use of Nexviazyme with the combination product Pombiliti (Opfolda). This exclusion aligns Nexviazyme criteria with the Pombiliti/Opfolda criteria and was approved in the 2Q 2024 update (P&T Approval Date = 05.24).

Non‑FDA approved indications that lack sufficient supporting documentation, or that are not addressed by the referenced formulary/off‑label policies, are not authorized for coverage. Providers seeking coverage for off‑label uses must follow the referenced off‑label/formulary policies and submit supporting clinical evidence as specified.

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Providers must obtain prior authorization before initiating nexviazyme (avalglucosidase alfa) and submit documentation confirming diagnosis of late-onset Pompe disease and the clinical tests used to establish the diagnosis per the policy criteria.

Prior authorization required for initiation and continuation per Health Plan processes.
Diagnosis must be confirmed by biochemical (enzyme assay demonstrating low GAA activity) and/or genetic (DNA) testing or other supported clinical evidence as outlined in the policy.

Billing Rule

Prior Authorization and Coding

Claims for avalglucosidase alfa-ngpt should use HCPCS code J0219 (Injection, avalglucosidase alfa-ngpt, 4 mg). Providers must obtain prior authorization per the Health Plan's standard processes prior to billing.

Use HCPCS J0219 for nexviazyme infusions.
Obtain prior authorization before submitting claims to avoid claim rejections.

Denial Risk

Coding-based Denial Risk

Inclusion or exclusion of codes in this policy does not guarantee coverage. Providers must reference current coding guidance and payer billing rules when submitting claims; incorrect or outdated coding may lead to denial.

Codes referenced are informational only; verify the most up-to-date professional coding resources before claim submission.
Coding errors or use of non-covered codes may result in denial of reimbursement.

Documentation Required

Required Documentation

Provider must submit supporting documentation (such as office chart notes, laboratory results, genetic testing reports, or other clinical information) demonstrating that the member meets all approval criteria.

Include enzyme assay results showing low GAA activity and/or DNA testing results when applicable.
Attach relevant clinical notes, baseline assessments, and any prior treatment history.

Note

Formulary / Off‑Label Routing

Formulary and off‑label routing requirements apply. If the requested use is on the formulary/PDL follow the formulary coverage rules; if not on formulary or represents an unlisted indication, follow the referenced non‑formulary or off‑label policies for the member's line of business.

For formulary requests, follow the relevant line-of-business no-coverage criteria/policy.
For non‑formulary or off‑label uses, follow the applicable non‑formulary or off‑label policies (see policy references for CP.CPA.190, HIM.PA.33, CP.PMN.255, CPCPA.190, HIMPA.103, CP.PMN.16, CP.CPA.09, HIMPA.154, CP.PMN.53 as applicable).

Note

Policy Requirements (History)

Policy history and requirements have changed over time; providers must follow the current policy criteria. Notable historical updates include narrowing covered diagnoses to late‑onset Pompe disease, requiring biochemical or genetic testing to confirm diagnosis, removing the endocrinologist prescriber requirement, and adding exclusions for concomitant use with specified therapies.

Policy effective date: 2021-08-06; subsequent updates include labeling-aligned criteria and periodic annual reviews.
Current clinical requirements (e.g., diagnostic testing, dosing limits, and exclusions) reflect the latest policy version — always reference the live policy for the operative criteria.

Initial Therapy Criteria

inv-20: Initial Therapy — Initial approval requirements and dosing

Initial approval requirements and dosing

Diagnostic confirmation: Diagnosis of late‑onset Pompe disease confirmed by one of: enzyme assay demonstrating low GAA activity; DNA testing; or increased lysosomal glycogen.

Required biochemical or genetic testing per policy history

No concurrent ERTs: Nexviazyme must not be prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda.

Concurrent therapy exclusion

Weight‑based dosing: For members weighing ≥30 kg: 20 mg/kg every 2 weeks; for members weighing <30 kg: 40 mg/kg every 2 weeks.30 kg

Dose and maximum dose statements in policy

Continuation Therapy Criteria

inv-22: Continuation Therapy — Requirements for continuation of therapy

Requirements for continuation of therapy

Prior criteria or current therapy: Member is currently receiving medication via Centene benefit or previously met initial approval criteria; continuity of care rules may apply.

Continuation eligibility

Demonstrated clinical benefit: Member is responding positively to therapy as evidenced by improvement or maintenance of disease manifestations (examples include improved or maintained forced vital capacity and improved or maintained 6MWT distance).

Examples: improved/maintained FVC, improved/maintained 6MWT

Step Therapy / Routing

When request is outside formulary/PDL or represents off‑label use	Action / Routing
Requested use is for a drug that is on the formulary (commercial, health insurance marketplace) or on the PDL (Medicaid)	Refer to the no‑coverage criteria policy for the relevant line of business: CP.CPA.190 (commercial), HIM.PA.33 (health insurance marketplace), or CP.PMN.255 (Medicaid). If the drug has had a recent label change within the last 6 months, follow the alternate routing rules in the referenced policies.
Requested use is for a drug that is NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid)	Refer to the non‑formulary policy for the relevant line of business: CP.CPA.190 (commercial), HIM.PA.103 (health insurance marketplace), or CP.PMN.16 (Medicaid).
Requested use (diagnosis, age, or regimen) is not specifically listed under the policy's 'Diagnoses/Indications for which coverage is NOT authorized' and does not meet the above formulary/non‑formulary routing	Refer to the off‑label use policy for the relevant line of business: CP.CPA.09 (commercial), HIM.PA.154 (health insurance marketplace), or CP.PMN.53 (Medicaid).

Policy revision topic	Summary of change / impact for providers
Removal of endocrinologist prescriber requirement	The policy removed the prior requirement that an endocrinologist be the prescriber — prescriber specialty is no longer required for coverage determinations (aligned with Lumizyme policy).
Updated diagnostic confirmation requirements	Diagnostic confirmation was updated to require biochemical or genetic testing to confirm Pompe diagnosis (examples: enzyme assay showing low GAA activity, DNA testing, or increased lysosomal glycogen) in alignment with FDA labeling.
Clarification of covered diagnosis	Covered diagnosis was narrowed to late‑onset Pompe disease only; providers should follow current policy criteria for diagnosis confirmation when submitting prior authorization requests.
Administrative and review history notes	Policy history entries document template updates, review cycles, and an added exclusion for concomitant use with Pombiliti‑Opfolda (2Q 2024). Providers should use the current policy criteria and exclusions when seeking authorization.

Site of Care and Infusion Details

Note

Monitor for infusion reactions and cardiorespiratory risk during administration

Infusions carry risks including severe hypersensitivity and infusion‑associated reactions and potential acute cardiorespiratory failure in susceptible patients; ensure appropriate monitoring and infusion setting decisions.

Be prepared to manage severe hypersensitivity and infusion‑associated reactions during administration.
Consider site‑of‑care monitoring based on patient risk.

Billing Rule

Bill infusions using J0219 and follow site‑of‑care billing rules

When billing for infusion of avalglucosidase alfa-ngpt, reference HCPCS code J0219 and comply with Health Plan site‑of‑care and billing requirements.

Use HCPCS code J0219 for infusion billing.
Follow the Health Plan's site‑of‑care billing rules when submitting claims.

OpenPayer

Clinical Policy: Avalglucosidase Alfa-ngpt (Nexviazyme)

Coverage Criteria

Coding and Dosing Details

Provider Actions and Requirements

Definitions

Initial Therapy Criteria

Continuation Therapy Criteria

Step Therapy / Routing

Site of Care and Infusion Details

Background