CurrentCignaPolicy IP0444

Enzyme Replacement Therapy - Elaprase (idursulfase)

This policy governs prior authorization and coverage criteria for Elaprase (idursulfase intravenous infusion) for treatment of Mucopolysaccharidosis type II (Hunter syndrome) for members covered by Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyEnzyme Replacement Therapy - Elaprase (idursulfase)

Policy CodePolicy IP0444

Change TypeDosing addedcriteria unchanged

Effective Date

Next Review Date

Key ActionObtain prior authorization and document diagnostic confirmation (enzyme assay or molecular genetic test) and specialist prescriber criteria; approvals are for 1 year.

SourceLink

POLICY UPDATE CHANGES

Added dosing.

No criteria changes were made during the revision.

1 yearinitial approval duration

0.5 mg/kgmax single dose

weeklymax dosing frequency

J1743covered code

Coverage Criteria for Elaprase (idursulfase)

Policy Summary

PayerCigna

PolicyEnzyme Replacement Therapy - Elaprase (idursulfase)

Policy CodePolicy IP0444

Change TypeDosing addedcriteria unchanged

Effective Date

Next Review Date

Key ActionObtain prior authorization and document diagnostic confirmation (enzyme assay or molecular genetic test) and specialist prescriber criteria; approvals are for 1 year.

SourceLink

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FDA-Approved Indication: Patient has Mucopolysaccharidosis Type II (Hunter syndrome)

Diagnostic confirmation and prescriber: A) Diagnosis established by ONE of: i) laboratory test demonstrating deficient iduronate-2-sulfatase activity in leukocytes, fibroblasts, serum, or plasma; OR ii) molecular genetic test demonstrating an iduronate-2-sulfatase (IDS) gene variant; AND B) Elaprase is prescribed by or in consultation with a geneticist, endocrinologist, metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders.

Approval duration: 1 year. Dosing must not exceed 0.5 mg/kg IV no more frequently than once weekly.

Coding and Dosing Information

Considered medically necessary when criteria metHCPCSCovered

J1743

Injection, idursulfase, 1 mg

inv-05: Dose per administration

Dose per administrationEach dose must not exceed 0.5 mg/kg administered intravenously.

Unit of measuremg per kilogram of body weight (mg/kg)

Administration routeIntravenous (IV)

inv-06: Dosing interval

Dosing intervalAdminister no more frequently than once weekly.

Maximum frequencyOnce every 7 days

Relation to dosing limitInterval requirement applies in conjunction with maximum dose of 0.5 mg/kg per administration.

Diagnostic Testing and Covered Indications

Diagnostic genetic testing to confirm iduronate-2-sulfatase (IDS) gene variants for suspected MPS II

A molecular genetic test demonstrating an IDS gene variant satisfies diagnostic criteria when used to confirm suspected MPS II.

Diagnostic genetic testing: Molecular genetic testing that identifies a pathogenic or likely pathogenic variant in the iduronate-2-sulfatase (IDS) gene that explains the clinical presentation of suspected MPS II.

A positive molecular genetic test is an accepted pathway to establish diagnosis for coverage of Elaprase; enzyme assay in leukocytes, fibroblasts, serum, or plasma is an alternative diagnostic pathway.

Provider Actions and Authorization Requirements

Prior Authorization

Prior authorization required — 1 year approvals; out‑of‑range doses reviewed

Prior authorization is required for benefit coverage of Elaprase. Approvals are provided for 1 year when the patient meets the diagnostic and prescriber specialty criteria; requests for doses outside the dosing established in the policy will be considered case‑by‑case by a clinician.

Approve for 1 year if patient meets BOTH diagnostic confirmation (enzyme assay or molecular genetic test) and prescriber specialty requirements.
Requests for doses outside established dosing (≤0.5 mg/kg IV no more frequently than once weekly) are reviewed case‑by‑case.

Step Therapy

No step therapy sequence specified

There is no required step‑therapy sequence. Coverage decisions are based on documented diagnostic confirmation and that Elaprase is prescribed by or in consultation with an appropriate specialist.

No prior medication or treatment sequence required before approval.
Approval contingent on meeting diagnostic and specialist prescriber criteria.

Documentation Required

Required documentation — diagnostic confirmation and specialist prescriber

Documentation must show diagnostic confirmation of MPS II (either deficient iduronate‑2‑sulfatase enzyme activity in leukocytes, fibroblasts, serum, or plasma, or a molecular genetic test demonstrating an IDS gene variant) and that Elaprase is prescribed by or in consultation with an appropriate specialist.

Include laboratory evidence of deficient iduronate‑2‑sulfatase activity OR molecular genetic test demonstrating an iduronate‑2‑sulfatase (IDS) gene variant.
Document the prescribing clinician’s specialty or consultation with a geneticist, endocrinologist, metabolic disorder sub‑specialist, or lysosomal storage disorders specialist.

Denial Risk

Denials possible for missing/incorrect codes or lack of prior authorization

Claims submitted without covered diagnosis or procedure codes or not submitted in accordance with the applicable Coverage Policy will be denied; prior authorization is required for benefit coverage of Elaprase.

Use the most appropriate covered diagnosis and procedure codes when billing; services billed for conditions not covered by this policy will be denied.
Prior authorization must be obtained for benefit coverage.

Note

Diagnostic genetic testing accepted; counseling not explicitly required

Policy requires diagnosis by molecular genetic testing as one of the acceptable diagnostic pathways (molecular genetic test demonstrating an iduronate‑2‑sulfatase gene variant). The policy does not explicitly require pre‑ or post‑test genetic counseling in the text.

A molecular genetic test demonstrating an IDS gene variant satisfies diagnostic criteria for coverage.
Pre/post‑test counseling is not specified as a requirement in the policy language.

Note

Prescriber specialty requirement — specialist or consult required

Elaprase must be prescribed by or in consultation with a geneticist, endocrinologist, metabolic disorder sub‑specialist, or a physician who specializes in the treatment of lysosomal storage disorders; this specialty requirement is necessary for approval and ongoing monitoring.

Specialist prescriber requirement applies at initial approval and for extended approvals.
Because of the specialized skills required, approvals require prescribing by or consultation with the listed specialists.

Definitions

inv-12: Definitive diagnosis

Definitive diagnostic methodsDemonstration of deficient iduronate-2-sulfatase activity in leukocytes, fibroblasts, serum, or plasma.

Genetic confirmationIdentification of mutation(s) in the iduronate-2-sulfatase (IDS) gene via molecular genetic testing.

Diagnostic pathways accepted for coverageEither enzyme activity assay OR molecular genetic testing demonstrating an IDS gene variant satisfies diagnostic criteria for Elaprase coverage.

Specimen types for enzyme assayLeukocytes, fibroblasts, serum, or plasma.

Background

Mucopolysaccharidosis type II (Hunter syndrome) is an X-linked lysosomal storage disorder caused by iduronate-2-sulfatase deficiency resulting in accumulation of glycosaminoglycans; males are predominantly affected and disease severity varies. Definitive diagnosis is established by demonstrating deficient iduronate-2-sulfatase activity in leukocytes, fibroblasts, serum, or plasma, or by identifying a pathogenic variant in the iduronate-2-sulfatase (IDS) gene. Enzyme replacement therapy with Elaprase addresses the systemic manifestations of MPS II; treatment decisions and prescribing should be coordinated with specialists experienced in lysosomal storage disorders.

OpenPayer