CurrentCareSourcePolicy N/A

Naglazyme (galsulfase) — Prior Authorization and Coverage Criteria for MPS VI

Defines prior authorization and coverage criteria for Naglazyme (galsulfase) as enzyme replacement therapy for members with confirmed mucopolysaccharidosis VI (MPS VI); applies to medical benefit use in CareSource-covered populations (Arkansas PASSE).

Policy Summary

PayerCareSource

PolicyNaglazyme (galsulfase) — Coverage criteria for MPS VI

Policy CodePolicy N/A

Change TypeMaterial updates to renewal requirements

Effective Date07/01/2024

Next Review Date

Key ActionObtain prior authorization and submit baseline uGAG and specialist documentation; for renewal include reduced uGAG plus evidence of clinical improvement or stabilization.

SourceLink

POLICY UPDATE CHANGES

Pulmonary response (e.g., FVC or FEV1) was added as an option for renewal criteria.

Renewal requirements changed to require both reduction in uGAG and clinical response.

Policy created (new policy) on 07/09/2021.

1 mg/kgdosage allowed

6 monthsinitial approval

12 monthsrenewal approval

<10%diagnostic threshold

Coverage Criteria for Naglazyme (galsulfase)

Initial Authorization

Covered when ALL of the following are met for initial authorization:

Initial Authorization: Medication is prescribed by or in consultation with a geneticist, metabolic specialist, or pediatrician experienced in managing mucopolysaccharidoses; AND Member has a diagnosis of MPS VI confirmed by at least one of: (a) ASB enzyme activity < 10% of the lower limit of normal with normal activity of a second sulfatase (to exclude multiple sulfatase deficiency), and/or (b) molecular genetic analysis identifying mutations of the ARSB gene; AND Chart notes include baseline urinary GAG (uGAG) levels showing elevated dermatan sulfate; AND Dosage: 1 mg/kg IV once weekly.

If all the above requirements are met, approve for 6 months.

Reauthorization

Reauthorization is covered when ALL of the following are met:

Reauthorization: Chart notes show reduced urinary GAG (uGAG) excretion; AND Chart notes show improvement or stabilized signs and symptoms such as improved endurance (e.g., walk test or stair climb) OR improved pulmonary function (e.g., FVC or FEV1).

Policy Summary

PayerCareSource

PolicyNaglazyme (galsulfase) — Coverage criteria for MPS VI

Policy CodePolicy N/A

Change TypeMaterial updates to renewal requirements

Effective Date07/01/2024

Next Review Date

Key ActionObtain prior authorization and submit baseline uGAG and specialist documentation; for renewal include reduced uGAG plus evidence of clinical improvement or stabilization.

SourceLink

On This Page

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If all the above requirements are met, approve for an additional 12 months.

CareSource considers Naglazyme (galsulfase) not medically necessary for the treatment of conditions that are not listed in this document. For indications other than MPS VI (Maroteaux-Lamy syndrome), providers should follow the health plan’s Off-Label policy and obtain prior authorization as required.

Use of Naglazyme for any diagnosis or purpose other than the covered indication(s) specified in this policy is considered not medically necessary. Requests for coverage for alternate indications must be submitted under the Off-Label policy and will be evaluated on that basis.

Initial Therapy Requirements

Initial Therapy

Covered when ALL of the following are met for initial treatment:

Initial: Medication prescribed by or in consultation with an appropriate specialist (geneticist, metabolic specialist, or pediatrician experienced with mucopolysaccharidoses); AND Diagnosis of MPS VI confirmed by enzyme assay (ASB activity < 10% of lower limit of normal with normal second sulfatase) or by molecular genetic testing identifying ARSB mutations; AND Baseline urinary GAG (uGAG) demonstrates elevated dermatan sulfate; AND Dosing: 1 mg/kg IV once weekly.

Approve for 6 months if criteria met.

Continuation / Reauthorization Criteria

Continuation Therapy

Reauthorization/continuation criteria:

Continuation: Documentation of reduced urinary GAG (uGAG) excretion AND clinical improvement or stabilization demonstrated by endurance measures (e.g., walk test or stair climb) OR pulmonary response (e.g., improved FVC or FEV1).

If met, approve for 12 months.

Coding and Diagnostic Thresholds

Drug name (informational)NDC | mixed

galsulfase

Naglazyme (drug name as listed)

inv-06: ASB enzyme activity threshold

ASB enzyme activity thresholdLess than 10% of the lower limit of normal (with normal activity of a second sulfatase to exclude Multiple Sulfatase Deficiency)

Clinical contextUsed to confirm diagnosis of MPS VI (Maroteaux-Lamy syndrome)

Diagnostic roleOne of the accepted methods for initial authorization of Naglazyme

Provider Actions and Documentation Requirements

Prior Authorization

Prior Authorization Required — Naglazyme coverage

Prior authorization is required. Naglazyme (galsulfase) is covered as a medical benefit when all clinical criteria are met and prior authorization has been obtained.

Benefit type: Medical
Prior authorization required before initiation of therapy
If criteria are met, initial approval duration: 6 months; reauthorization duration: 12 months

Step Therapy

Step therapy

There are no step therapy requirements for Naglazyme. Providers do not need to trial alternative therapies prior to requesting prior authorization.

Documentation Required

Required documentation

Providers must submit documentation to support the medical necessity request. Required documentation includes baseline and follow-up clinical and laboratory data as specified below.

Chart notes from prescribing provider (geneticist, metabolic specialist, or pediatrician experienced with mucopolysaccharidoses)
For initial request: evidence of confirmed MPS VI by either ASB enzyme activity < 10% of the lower limit of normal with a normal second sulfatase to exclude Multiple Sulfatase Deficiency, and/or molecular genetic testing showing ARSB mutations
Baseline urinary glycosaminoglycan (uGAG) levels demonstrating elevated dermatan sulfate for initial authorization
Dose and administration documentation: prescribed dose 1 mg/kg IV weekly
For reauthorization: chart notes documenting reduced uGAG excretion and clinical improvement or stabilization (e.g., improved endurance such as walk test or stair climb, or improved pulmonary function such as FVC or FEV1)

Denial Risk

Triggers for denial — common reasons requests are denied

Requests that do not meet the documented criteria are at high risk for denial. Common reasons for denial include missing or incomplete diagnostic confirmation, lack of required baseline or follow-up uGAG data, absence of documentation showing clinical benefit on reauthorization, and prescribing by a provider without appropriate specialty involvement.

No confirmed diagnosis of MPS VI (missing enzyme assay or genetic testing)
ASB enzyme activity not documented as < 10% or lack of second sulfatase testing to exclude Multiple Sulfatase Deficiency
Baseline uGAG with dermatan sulfate not provided
For reauthorization: no evidence of reduced uGAG excretion or no documented clinical improvement/stabilization
Dose exceeds the stated quantity limit (1 mg/kg IV weekly) or missing dose/admin information
Requests for indications not listed in the policy (off-label) without appropriate Off-Label policy justification

Definitions and Diagnostic Confirmation

inv-12: Diagnostic confirmation of MPS VI

Enzyme assay criterionASB enzyme activity < 10% of the lower limit of normal AND activity of a second sulfatase is normal (to exclude Multiple Sulfatase Deficiency)

Genetic testing criterionMolecular genetic analysis identifying pathogenic variants (mutations) in the ARSB gene

Acceptable confirmationDiagnosis may be confirmed by either the enzyme assay above or by ARSB molecular genetic testing

Background

Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is a rare lysosomal storage disorder caused by deficient arylsulfatase B activity due to pathogenic variants in the ARSB gene, which leads to accumulation of dermatan sulfate. Naglazyme (galsulfase) is an enzyme replacement therapy intended to replace the deficient enzyme and improve functional outcomes such as walking and stair-climbing capacity.

Step Therapy

Step	Requirement
No step therapy specified in this policy.	No step therapy requirements are defined for Naglazyme (galsulfase) in this policy.

Quantity Limits and Dosing

inv-16: Naglazyme (galsulfase) dosing

Standard dosing1 mg/kg intravenous once weekly

Quantity limitDosage allowed/quantity limit specified as 1 mg/kg per week

Approval duration with correct dosingIf initial criteria met (including dosing), approve for 6 months

Site of Care

Note

No infusion center/site-of-care restrictions

No site-of-care limitations are specified for infusion of Naglazyme in this policy.