Pharmacy Prior Authorization Criteria

Mepsevii (vestronidase alfa-vjbk)

Indications for Prior Authorization

Mepsevii (vestronidase alfa-vjbk)

• For diagnosis of Mucopolysaccharidosis (MPS VII, Sly Syndrome)
  Indicated for the treatment of Mucopolysaccharidosis (MPS VII, Sly Syndrome) in pediatric and adult patients.

  Limitations of use: The effect of Mepsevii on the central nervous system manifestations of MPS VII has not been determined.

Criteria

Mepsevii

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Mucopolysaccharidosis (MPS VII, Sly Syndrome)

• Submission of medical records (e.g., chart notes) confirming diagnosis of Mucopolysaccharidosis VII (MPS VII, Sly syndrome)
AND
• Diagnosis is confirmed by one of the following:
• Submission of medical records (e.g., chart notes) confirming absence or deficiency of beta-glucuronidase (GUSB) enzyme activity in fibroblasts or leukocytes
OR
• Both of the following:
• Submission of medical records (e.g., chart notes) confirming presence of mutation in GUSB gene
AND
• Provider attests mutation was detected by a FDA-approved test or a test performed at a facility approved by CLIA.

Mepsevii

Prior Authorization (Reauthorization)

Length of Approval: 24 Month(s)
For diagnosis of Mucopolysaccharidosis (MPS VII, Sly Syndrome)

• Patient demonstrates positive clinical response to therapy

P & T Revisions

1970-01-01, 2026-07-06, 2026-06-03, 2025-04-30, 2024-04-26, 2023-10-03, 2023-05-03, 2022-03-28, 2021-09-27, 2021-05-20, 2020-05-14

References

1. Mepsevii Prescribing Information. Ultragenyx Pharmaceutical Inc. Novato CA. December 2020.
2. Mucopolysaccharidoses: Clinical features and diagnosis, Sheldon L Kaplan, MD. UpToDate, September 2024, Accessed June 2026
3. Mucopolysacchardidosis type 7, Genetic and Rare Diseases Information Center, National Center for Advancing Translational Sciences, National Institutes of Health. Accessed June 2026

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Revision History

• 2026-07-06: Guideline update to include confirmation of disease as part of criteria.
• 2026-06-03: 2026 Annual Review: No criteria changes.
• 2025-04-30: Annual review: No criteria changes.
• 2024-04-26: Annual review: No criteria changes.
• 2023-10-03: Program update to standard reauthorization language. No changes to clinical intent
• 2023-05-03: Annual review: Initial authorization approval duration updated to 12 months. New reauthorization section added.
• 2022-03-28: 2022 Annual Review - No changes to criteria
• 2021-09-27: Annual review
• 2021-05-20: Annual review
• 2020-05-14: Annual Review - No Changes

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