Centene galsulfase (Naglazyme) Coverage Update

Coverage Criteria

Initial Therapy

Covered when ALL of the following are met:

Initial MPS VI approval: 1) Diagnosis of MPS VI (Maroteaux-Lamy syndrome) confirmed by either: (a) enzyme assay demonstrating a deficiency in N-acetylgalactosamine 4-sulfatase (arylsulfatase B) activity, OR (b) DNA testing; 2) Age ≥ 3 months; 3) Documentation of member's current weight (in kg); 4) Dose does not exceed 1 mg/kg per week.

Continuation Therapy

Covered when ALL of the following are met:

Continuation MPS VI: 1) Member is currently receiving medication via Centene benefit or previously met initial approval criteria OR member is currently receiving medication and enrolled in a state/product with continuity of care; 2) Member is responding positively to therapy as evidenced by improvement in the individual's MPS VI manifestation profile (examples include 12-minute walking test distance, 3-minute stair climb rate, endurance, vision, respiratory infections, breathing problems/sleep apnea, high blood pressure, joint stiffness, height and weight, hepatomegaly, or splenomegaly); 3) Documentation of member's current weight (in kg); 4) If request is for a dose increase, new dose does not exceed 1 mg/kg per week.

Examples of response referenced from Appendix D.

Other Indications / Off-label routing

For other requested uses not listed in the coverage sections:

Other Indications: 1) If the drug has undergone a label change within the last 6 months that is not yet reflected in this policy, refer to the applicable formulary/no-coverage or non-formulary policy for the relevant line of business (see CP.CPA.190, HIM.PA.33, CP.PMN.255, HIM.PA.103, CP.PMN.16). OR 2) If the requested use (diagnosis, age, dosing) is not specifically listed and criterion 1 does not apply, refer to the off-label use policy for the relevant line of business (CP.CPA.09, HIM.PA.154, CP.PMN.53).

Follow formulary/non-formulary or off-label policies as routing guidance.

Coverage with criteria

Policy covers galsulfase when clinical criteria and documentation requirements are met per the Health Plan clinical policy.

Documentation and authorization: 1) Prior authorization obtained per Health Plan procedures (HCPCS J1458 referenced in policy coding sections); 2) Documentation includes member's current weight for dose calculation and clinical rationale consistent with policy; 3) Initial approvals are for 12 months (per updated initial authorization).12 months

Specific medical necessity criteria and response examples are referenced in the policy and Appendix D.

Requests for uses that are not FDA‑approved and are not specifically addressed in this policy are not authorized unless the provider supplies sufficient documentation of efficacy and safety consistent with the applicable off‑label use policies (Commercial: CP.CPA.09; HIM/ICHRA: HIM.PA.154; Medicaid: CP.PMN.53) or evidence of coverage documents.

There are no additional explicit exclusion conditions specified in the cited policy text beyond standard limitations in coverage documents. Coverage determinations remain subject to the terms, conditions, exclusions, and limitations of the member's evidence of coverage and applicable Health Plan administrative policies.

Use of galsulfase for non‑FDA approved indications without adequate supporting evidence is not authorized. Providers must follow the referenced off‑label use policies and provide documentation demonstrating efficacy and safety to pursue coverage for off‑label uses.

Coding entries in this policy are provided for informational purposes only; inclusion or exclusion of any code does not guarantee coverage. The cited excerpts do not list specific conditions that are designated as not medically necessary in this section; providers should confirm coding and coverage applicability against current professional coding guidance and the member's coverage documents.

Coding and Dosing

HCPCS CodesHCPCS

J1458

Injection, galsulfase, 1 mg

HCPCS CodesHCPCS

J1458

Injection, galsulfase, 1 mg

Maximum dose — galsulfase (Naglazyme)

Maximum dose1 mg/kg per week (IV)

Dosing frequencyOnce weekly IV administration

Dose limit applicationApplies to initial and continued therapy; dose increases must not exceed 1 mg/kg/week

Initial approval duration

Initial approval duration12 months

Commercial guidanceCommercial approvals historically 6 months or to member renewal date; updated to 12 months per 2Q2026 review

Line-of-business note

Provider Actions and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required. Providers must obtain prior authorization per clinical policy (refer to HCPCS J1458 for galsulfase) and submit diagnostic confirmation, age, current weight, dosing, and other supporting information to show criteria are met.

Diagnostic confirmation (enzyme assay or DNA testing) required for MPS VI
Age requirement (≥ 3 months) must be documented
Current weight (kg) required for dose calculation — dose limit: 1 mg/kg per week
Include proposed dose and rationale for dosing or dose increases

Prior Authorization

Non‑FDA Indications / Policy Routing for Other Indications

Requests for indications not FDA‑approved or not listed in this policy will be considered only with sufficient supporting evidence of efficacy and safety per the applicable off‑label use policy. If the requested use is outside the listed criteria, route the request per the formulary/off‑label policies for the member's line of business.

Non‑FDA indications require documentation consistent with off‑label policy for the relevant line of business

Initial Therapy Criteria

Initial

Initial therapy coverage criteria

Initial therapy: Diagnosis of MPS VI confirmed by enzyme assay demonstrating arylsulfatase B deficiency or by DNA testing; age ≥ 3 months; documentation of current weight (kg); dose ≤ 1 mg/kg IV once weekly.

Approval durations vary by line of business.

Initial Therapy

Initial authorization updated (operational changes noted)

Initial authorization: Initial approval duration is 12 months; documentation must include current weight for dose calculation and clinical rationale per policy.12 months

Initial approval duration was updated from 6 months to 12 months during 2Q 2026 review.

Continuation / Continued Therapy Criteria

Continuation

Criteria for continued coverage

Continued therapy: Member currently receiving drug or previously met initial criteria and demonstrating positive response as evidenced by improvement in MPS VI manifestations; documentation of current weight; dose limits apply (≤ 1 mg/kg/week).

Positive response examples include walk/stair tests, endurance, respiratory, vision, cardiac and organomegaly improvements (see Appendix D).

Continued Therapy

Continued therapy requirements reference examples of positive treatment response (added from Appendix D)

Continued therapy response: Documentation of positive treatment response per examples referenced in Appendix D (e.g., improvement in 12-minute walk distance, 3-minute stair climb rate, endurance, vision, respiratory infections, breathing problems/sleep apnea, blood pressure, joint stiffness, growth, hepatomegaly/splenomegaly).

Examples were incorporated into the continued therapy section during policy update.

Step Therapy

Step	Requirement
1	If the drug has undergone a label change within the last 6 months (new indication, age expansion, or dosing change) not yet reflected in this policy, follow the no‑coverage or non‑formulary policy for the applicable line of business: CP.CPA.190 (Commercial), HIM.PA.33 or HIM.PA.103 (Marketplace/ICHRA), or CP.PMN.255 or CP.PMN.16 (Medicaid).
2	If the requested use (diagnosis, age, dosing) is not listed in section III and criterion 1 does not apply, route the request to the off‑label use policy for the relevant line of business: CP.CPA.09 (Commercial), HIM.PA.154 (Marketplace/ICHRA), or CP.PMN.53 (Medicaid).

Step	Requirement
0	No specific step therapy requirements are specified in the policy chunks provided. If needed, follow applicable formulary, non‑formulary, or off‑label policies as directed by the Health Plan procedures.

Quantity Limits

galsulfase (Naglazyme) — dosing & product availability

Dosing basisPer mg dosing calculated from patient weight (1 mg/kg/week)

PresentationSupplied as 5 mg/5 mL vials

Quantity policyVials may be vialized as needed to meet weight-based mg dosing per week

Site of Care

Note

IV infusion once weekly; site of care not restricted

Galsulfase is administered intravenously once weekly; this policy does not restrict the specific site of care (infusion center administration is documented).

Dosing regimen: 1 mg/kg IV once weekly
Site of care not restricted in this document

Background

Mucopolysaccharidosis VI (MPS VI; Maroteaux‑Lamy syndrome) is a lysosomal storage disorder caused by deficiency of arylsulfatase B (N‑acetylgalactosamine 4‑sulfatase). The resulting enzyme deficiency leads to impaired degradation of glycosaminoglycans with progressive clinical manifestations including reduced endurance, impaired walking and stair‑climb ability, organomegaly, respiratory compromise, vision problems, and other somatic features. Galsulfase (Naglazyme) is an enzyme replacement therapy indicated to treat MPS VI and may improve walking and stair‑climbing capacity and other disease manifestations; clinical response can be assessed using measures such as walk/stair‑climb tests, endurance, respiratory and vision findings, cardiac and organomegaly changes.

Definitions

MPS VI — definition and diagnostic confirmation

DefinitionMPS VI (Maroteaux-Lamy syndrome): a lysosomal storage disorder caused by deficiency of arylsulfatase B (N-acetylgalactosamine 4-sulfatase)

Diagnostic confirmationConfirmed by enzyme assay demonstrating deficiency of N-acetylgalactosamine 4-sulfatase (arylsulfatase B) activity, OR DNA testing

Clinical manifestationsMay include impaired endurance, walking/stair-climb ability, vision and respiratory problems, organomegaly; improvements measurable by 12-minute walk and 3-minute stair climb tests

Definition — 'Health Plan'

'Health Plan' definition'Health Plan' means a health plan that has adopted this clinical policy and that is operated or administered, in whole or in part, by Centene Management Company, LLC, or any of such health plan's affiliates.

Policy applicability

OpenPayer

Galsulfase (Naglazyme) (Clinical Policy)