Subject: Naglazyme SGM 2056-A P2025

Drug

NAGLAZYME  (galsulfase)

Policy:

Indications

The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy.

FDA-Approved Indication¹

Naglazyme is indicated for patients with Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome). Naglazyme has been shown to improve walking and stair-climbing capacity.

All other indications are considered experimental/investigational and not medically necessary.

Documentation

Submission of the following information is necessary to initiate the prior authorization review:

• Initial requests: N-acetylgalactosamine-4-sulfatase (arylsulfatase B) enzyme assay or genetic testing results supporting diagnosis.
• Continuation requests: chart notes documenting a clinically positive response to therapy, which shall include improvement, stabilization, or slowing of disease progression.

Prescriber Specialties

This medication must be prescribed by or in consultation with a physician who specializes in the treatment of metabolic disease and/or lysosomal storage disorders.

Coverage Criteria

Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome)^1,2

Authorization of 12 months may be granted for treatment of MPS VI (Maroteaux-Lamy syndrome) when the diagnosis of MPS VI was confirmed by enzyme assay demonstrating a deficiency of N-acetylgalactosamine-4-sulfatase (arylsulfatase B) enzyme activity or by genetic testing.

Continuation of Therapy

Authorization of 12 months may be granted for continued treatment in members requesting reauthorization for an indication listed in the Coverage Criteria section who have a clinically positive response to therapy, which shall include improvement, stabilization, or slowing of disease progression.

Place of Service:

Outpatient

The above policy is based on the following references:

1. Naglazyme [package insert]. Novato, CA: BioMarin Pharmaceutical Inc.; September 2024.
2. Akyol, M.U., Alden, T.D., Amartino, H. et al. Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance. Orphanet J Rare Dis 14, 118 (2019).

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

July 22, 2025