CurrentCignaPolicy IP0441

Eliglustat (Cerdelga) for Gaucher disease type 1 — Medical Necessity and Coverage Criteria

Defines Cigna's medical necessity, authorization, and coverage stance for eliglustat (Cerdelga) for treatment of Gaucher disease type 1 in members whose benefits are administered by Cigna Companies.

Policy Summary

PayerCigna

PolicyEliglustat (Cerdelga) for Gaucher disease type 1

Policy CodePolicy IP0441

Change TypeSelected revision with material updates

Effective DateJul 15, 2025

Next Review DateJul 15, 2025

Key ActionObtain prior authorization; approvals (initial and reauthorization) are for 12 months when criteria are met.

SourceLink

POLICY UPDATE CHANGES

Removed criterion 'Individual is age 18 years or older'.

Updated laboratory requirement wording to 'demonstration of deficient beta-glucocerebrosidase activity in leukocytes or fibroblasts.'

Updated genetic confirmation wording to 'Confirmation of molecular genetic test documenting biallelic pathogenic glucocerebrosidase (GBA) gene variants.'

Specified CYP2D6 metabolizer status must be 'as detected by an approved test'.

Reauthorization criterion rephrased to state continuation is medically necessary when initial criteria are met AND there is documentation of beneficial response.

Added 'Concomitant use with other approved therapies for Gaucher disease' to Conditions Not Covered.

12 monthsapproval duration

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

EM/IM/PMCYP2D6 categories

12/1/2024selected revision date

Coverage Criteria for Eliglustat (Cerdelga)

Initial Therapy — Eliglustat (Cerdelga)

Eliglustat (Cerdelga) is considered medically necessary when ALL of the following are met for Gaucher disease type 1:

Gaucher Disease Type 1 overall: All subcriteria A–D must be satisfied

A. Diagnostic confirmation: There is documentation of EITHER: (i) demonstration of deficient beta-glucocerebrosidase activity in leukocytes or fibroblasts OR (ii) confirmation of molecular genetic test documenting biallelic pathogenic glucocerebrosidase (GBA) gene variants.

Supported by enzyme assay or molecular genetic testing.

B. CYP2D6 metabolizer status: The individual is ONE of the following as detected by an approved test: CYP2D6 extensive metabolizer (EM), CYP2D6 intermediate metabolizer (IM), or CYP2D6 poor metabolizer (PM).

Metabolizer status must be determined by an approved test.

C. Prescriber: Medication is prescribed by, or in consultation with, a geneticist, endocrinologist, metabolic disorder subspecialist, or a physician who specializes in the treatment of Gaucher disease or related disorders.

D. Dosing and care: Dosage, frequency, duration of therapy, and site of care should be reasonable, clinically appropriate, supported by evidence-based literature, and adjusted based upon severity, alternative available treatments, and previous response to therapy. Receipt of sample product does not satisfy criteria for coverage.

Continuation Therapy / Reauthorization

Continuation of Eliglustat (Cerdelga) is considered medically necessary when ALL of the following are met:

Reauthorization: Initial medical necessity criteria are still met AND there is documentation of beneficial response.

Approval duration: 12 months.

Eliglustat (Cerdelga) is not medically necessary for any indication other than treatment of Gaucher disease type 1. Conditions not covered include uses outside this indication; the list in the policy may be updated as new published data become available. Claims submitted for uses not covered under this policy may be denied.

Concomitant use of eliglustat with other therapies approved for Gaucher disease is considered not medically necessary. Examples of other approved Gaucher disease treatments cited in the policy include Cerezyme (imiglucerase), Elelyso (taliglucerase alfa), Vpriv (velaglucerase alfa), and Zavesca (miglustat). The policy notes that concomitant use has not been evaluated and has been added to the Conditions Not Covered.

Coding and Authorization Duration

No specific CPT/HCPCS/ICD-10 codes listed in policymixed

No codes listed

inv-06: Authorization Duration

Initial approval length12 months

Reauthorization approval length12 months

Reason for durationApproval durations align with prior authorization policy when criteria are met

Provider Requirements and Billing Guidance

Prior Authorization

Prior Authorization Required

Prior authorization required. Approvals are granted for up to 12 months when medical necessity criteria are met.

Initial and reauthorization approval duration: 12 months
Prior authorization required before initiation of therapy

Step Therapy

Step Therapy / Alternative Treatments

No step therapy is required prior to initiation of eliglustat. Concomitant use with other approved Gaucher disease therapies is not recommended and is considered not medically necessary.

No step therapy required
Concomitant use with other approved Gaucher disease therapies (e.g., imiglucerase, taliglucerase alfa, velaglucerase alfa, miglustat) is considered not medically necessary

Background

Gaucher disease type 1 is caused by deficiency of the lysosomal enzyme β‑glucocerebrosidase, which leads to accumulation of glucosylceramide in macrophages and the multisystem manifestations of the disease. Eliglustat (Cerdelga) is an oral glucosylceramide synthase inhibitor used as substrate reduction therapy for the long‑term treatment of adults with Gaucher disease type 1. Suitability and dosing of eliglustat depend on the individual’s CYP2D6 metabolizer status (extensive, intermediate, or poor) as detected by an approved test; molecular or enzymatic confirmation of Gaucher disease (enzyme assay showing deficient beta‑glucocerebrosidase activity in leukocytes or fibroblasts, or genetic testing documenting biallelic pathogenic GBA variants) is required for coverage consideration.

Definitions and Key Terms

inv-13: CYP2D6 metabolizer status

Accepted CYP2D6 phenotypesExtensive metabolizer (EM); Intermediate metabolizer (IM); Poor metabolizer (PM)

Test requirementMetabolizer status must be determined by an approved (FDA-cleared) test

Relevance to therapyEliglustat suitability and dosing depend on CYP2D6 metabolizer phenotype

Source guidancePolicy requires one of the listed phenotypes as detected by an approved test for medical necessity

inv-14: GBA biallelic pathogenic variants

DefinitionBiallelic pathogenic variants in the glucocerebrosidase (GBA) gene—pathogenic variants present in both alleles

Policy Summary

PayerCigna

PolicyEliglustat (Cerdelga) for Gaucher disease type 1

Policy CodePolicy IP0441

Change TypeSelected revision with material updates

Effective DateJul 15, 2025

Next Review DateJul 15, 2025

Key ActionObtain prior authorization; approvals (initial and reauthorization) are for 12 months when criteria are met.

SourceLink

On This Page

Documentation Required

Required Diagnostic and Prescriber Documentation

Documentation of diagnosis and prescriber qualifications is required. Provide laboratory or genetic confirmation and evidence of specialist involvement.

Diagnostic evidence: Either (a) demonstration of deficient beta-glucocerebrosidase activity in leukocytes or fibroblasts OR (b) molecular genetic testing confirming biallelic pathogenic GBA gene variants
Metabolizer status: Documentation from an approved test showing the individual is a CYP2D6 extensive (EM), intermediate (IM), or poor metabolizer (PM)
Prescriber: Medication must be prescribed by, or in consultation with, a geneticist, endocrinologist, metabolic disorder subspecialist, or a physician who specializes in treatment of Gaucher disease or related disorders
Clinical documentation: Relevant clinical notes supporting diagnosis, prior therapies, and rationale for eliglustat; receipt of sample product does not satisfy documentation requirements

Billing Rule

Coding / Claims Submission

Claims must be submitted with appropriate covered diagnosis and procedure codes. Claims submitted without covered codes or with services for indications not covered by this policy will be denied.

Claims without covered diagnosis or procedure codes will be denied as not covered
Use the most appropriate codes effective on the date of service

Denial Risk

Not Medically Necessary Uses

Use of eliglustat for indications other than Gaucher disease type 1 is considered not medically necessary. Concomitant use with other approved Gaucher disease therapies is also considered not medically necessary.

Not medically necessary for indications other than Gaucher disease type 1
Concomitant use with other approved Gaucher disease therapies (e.g., Cerezyme, Elelyso, Vpriv, Zavesca) is not medically necessary

Confirmation by molecular genetic testing documenting biallelic pathogenic GBA gene variants

Role in diagnosisRequired as one option for diagnostic confirmation of Gaucher disease type 1 for medical necessity

Alternative diagnostic optionCan be used in lieu of enzyme assay demonstrating deficient beta-glucocerebrosidase activity in leukocytes or fibroblasts