CurrentCignaPolicy IP0445

Aldurazyme

Defines prior authorization, medical necessity criteria, dosing limits, and coding for Aldurazyme (laronidase IV) for treatment of Mucopolysaccharidosis Type I (MPS I) under Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyAldurazyme

Policy CodePolicy IP0445

Change TypeAnnual revision; dosing added

Effective DateJul 15, 2025

Next Review Date

Key ActionSubmit prior authorization with laboratory evidence of deficient α-L-iduronidase activity or molecular genetic proof of biallelic IDUA variants and documentation of specialty prescriber consultation.

POLICY UPDATE CHANGES

Added dosing limits stating each dose must not exceed 0.58 mg/kg and frequency no more than once weekly.

Annual Revision: Mucopolysaccharidosis Type I wording adjusted; removed 'ONE of the following forms: Severe MPS I or Attenuated.'

1Covered Indication (MPS I)

1Covered HCPCS code

1Prior Authorization Required

2Diagnostic pathways

Coverage stance: Covered with criteria for Aldurazyme (laronidase) for Mucopolysaccharidosis Type I (MPS I). This policy defines prior authorization and medical necessity criteria, dosing limits, and coding for Aldurazyme under Cigna-administered health benefit plans. The policy effective date is 2025-07-15 and the last review/effective entry is recorded as 2025-07-15.

FDA-Approved Indication (Initial Approval)

Approve for 1 year if the patient meets BOTH of the following (A and B):

ALL of the following

Diagnosis: Mucopolysaccharidosis Type I (Hurler Syndrome, Hurler-Scheie Syndrome, or Scheie Syndrome).
Establish diagnosis by ONE of the following
- Laboratory test demonstrating deficient α-L-iduronidase activity in leukocytes, fibroblasts, plasma, or serum.
- Molecular genetic test demonstrating biallelic pathogenic or likely pathogenic α-L-iduronidase (IDUA) gene variants.
Prescriber requirement: Aldurazyme is prescribed by or in consultation with a geneticist, endocrinologist, a metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders.

Dosing Criteria / Limits

Dosing constraints that must be met for coverage:

ALL of the following

Each dose must not exceed 0.58 mg/kg administered intravenously.<= 0.58 mg/kg
Dosing frequency: no more frequently than once weekly.No more frequently than once weekly

Billing Rule

Dose billing and limits

Claims must reflect established dosing limits. Doses billed that exceed the policy limits will be reviewed on a case-by-case basis by a clinician.

J1931

Continuation Therapy Criteria

Continuation / Extended Approval

Extended approvals permitted if patient continues to meet criteria and dosing:

ALL of the following

Patient continues to meet the diagnostic criteria described in the FDA-Approved Indication section.
Patient continues to adhere to dosing limits (≤ 0.58 mg/kg per dose, no more than once weekly).<= 0.58 mg/kg; no more than once weekly

Unproven / Exclusions

Not Medically Necessary / Exclusions

Conditions not covered:

Use of Aldurazyme for any indication other than Mucopolysaccharidosis Type I (Hurler, Hurler-Scheie, Scheie) is considered not medically necessary.

Provider Actions

Prior Authorization

Prior authorization required

Prior authorization is required for benefit coverage of Aldurazyme. Approvals are recommended for those who meet the policy criteria and dosing and are granted for an initial duration of 1 year.

J1931

Documentation Required

Diagnostic documentation required

Submit laboratory evidence of deficient α-L-iduronidase activity in leukocytes, fibroblasts, plasma, or serum OR molecular genetic testing demonstrating biallelic pathogenic or likely pathogenic IDUA variants. Also include documentation of the specialty prescriber or consultation with the required specialist.

Documentation Required

Prescriber specialty requirement

Aldurazyme must be prescribed by or in consultation with one of the following: a geneticist, an endocrinologist, a metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders.

Billing Rule

Dose billing and limits

Billing must reflect doses of 0.58 mg/kg or less per administration and dosing frequency no more frequently than once weekly. Requests for doses outside these established dosing limits will be considered on a case-by-case basis by a clinician.

J1931

Clinical Evidence & Definitions

Key evidence and guidance sources include the FDA Prescribing Information: Aldurazyme intravenous infusion, Genzyme; Dec 2023 and the guideline reference Muenzer et al., Pediatrics 2009. Clinically relevant background points: enzyme replacement therapy with Aldurazyme does not cross the blood-brain barrier and is unlikely to improve cognitive or neurologic function; hematopoietic stem cell transplantation (HSCT) is indicated and preferred for severe forms in children <2 years who are cognitively intact; definitive diagnosis is established by demonstrating deficient α-L-iduronidase enzyme activity or by identifying biallelic pathogenic/likely pathogenic IDUA gene variants.

Definitive diagnosis of MPS IDemonstration of deficient α-L-iduronidase activity in fibroblasts, leukocytes, plasma, or serum, or molecular genetic evidence of biallelic pathogenic/likely pathogenic IDUA variants.

Dose per administration<= 0.58 mg/kg

FrequencyNo more frequently than once weekly

Background

Aldurazyme (laronidase) is an enzyme replacement therapy (human α-L-iduronidase) indicated for forms of Mucopolysaccharidosis Type I. It does not cross the blood-brain barrier and therefore is unlikely to improve cognitive or neurologic function. For severe MPS I in children under <2 years who are cognitively intact, hematopoietic stem cell transplantation (HSCT) is the preferred treatment because it can preserve intellectual development and improve survival; Aldurazyme may be appropriate for children who have already experienced cognitive decline or in those with severe somatic disease prior to HSCT, and for older patients regardless of cognitive status. Definitive diagnosis of MPS I is by demonstration of deficient α-L-iduronidase activity in fibroblasts, leukocytes, plasma, or serum, or by molecular genetic testing showing biallelic pathogenic/likely pathogenic IDUA variants.

Revision History

2024-08-01non-material_revision

Annual revision wording adjusted for Mucopolysaccharidosis Type I: removed phrasing listing disease forms ('ONE of the following forms: Severe MPS I or Attenuated'). No criteria changes were made.

2024-08-01material_change_recorded

Material change recorded adding dosing language: each Aldurazyme dose must not exceed 0.58 mg/kg and dosing frequency no more than once weekly. (Change recorded 8/1/2024; policy effective 2025-07-15).

2025-07-15policy_effective_dateLatest

Policy effective date for the updated coverage policy incorporating the dosing limits (≤ 0.58 mg/kg per dose, no more frequently than once weekly) and annual wording adjustments.

Policy Summary

PayerCigna

PolicyAldurazyme

Policy CodePolicy IP0445

Change TypeAnnual revision; dosing added

Effective DateJul 15, 2025

Next Review Date

On This Page

OpenPayer

Aldurazyme

Coverage Summary

Dosing Criteria / Limits

Continuation Therapy Criteria

Unproven / Exclusions

Applicable Codes

Provider Actions

Clinical Evidence & Definitions

Background

Revision History