CurrentCignaPolicy CNF340

Chenodiol Products Prior Authorization Policy - (CNF340)

Defines prior authorization requirements, clinical criteria, duration of approval, and non-covered uses for chenodiol products (Chenodal and Ctexli) for prescription benefit plans administered by Cigna companies.

Policy Summary

PayerCigna

PolicyChenodiol Products Prior Authorization Policy - (CNF340)

Policy CodePolicy CNF340

Change TypeClarified; Revised

Effective Date

Next Review Date

Key ActionPrior Authorization is recommended and approvals are provided for 1 year for approved indications; prescriber must be or consult a specialist appropriate to the condition.

SourceLink

POLICY UPDATE CHANGES

Policy name changed from 'Chenodal' to 'Chenodiol Products' and Ctexli tablets were added to the policy.

Annual revision with no criteria changes as of 08/28/2024 and 03/04/2026 reviews.

2Covered Indications

1Not Covered - Combination Therapy

1 yearApproval Duration (per indication)

Coverage Summary

Coverage stance: covered_with_criteria. Scope: policy defines prior authorization requirements, clinical criteria, duration of approval, and non-covered uses for chenodiol products (Chenodal and Ctexli) for prescription benefit plans administered by Cigna companies. Approval durations: approvals are provided for 1 year for each approved indication.

General Prior Authorization & Prescriber Requirement

Prior Authorization is recommended for prescription benefit coverage; approvals require specialist prescribing or consultation and are provided for the duration noted in each indication.

ALL of the following

Prior Authorization obtained
Medication is prescribed by or in consultation with a physician who specializes in the condition being treated (specialist required)

Initial Therapy Criteria

FDA-Approved Indication: Gallstones

Approve for 1 year if the patient meets ONE of the following (A or B):

ONE of

Patient has tried an ursodiol product
Patient is currently receiving an ursodiol product

FDA-Approved Indication: Cerebrotendinous Xanthomatosis (CTX)

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

ALL of the following

ONE of
- i: Molecular genetic test demonstrating a pathogenic variant in the cytochrome P450 27A1 (CYP27A1) gene
- ii: Laboratory test demonstrating elevated serum cholestanol levels
B: Medication is prescribed by or in consultation with a geneticist, neurologist, ophthalmologist, metabolic specialist who treats patients with cerebrotendinous xanthomatosis or a specialist who focuses in the treatment of cerebrotendinous xanthomatosis

Continuation Therapy Criteria

Continuation/approval duration policy: Prior Authorization is recommended and all approvals for approved indications are provided for 1 year. For CTX, diagnostic documentation (molecular genetic test showing a pathogenic CYP27A1 variant or laboratory evidence of elevated serum cholestanol) and documentation of specialist prescribing or consultation must be submitted for approval and renewal.

Prescriber requirement: the medication must be prescribed by or in consultation with an appropriate specialist; prior authorization renewal is expected to follow the same criteria and documentation requirements as initial approval.

Not Medically Necessary / Exclusions

Conditions Not Covered / Not Medically Necessary

Chenodiol products are considered not medically necessary for any other use(s) including:

Combination therapy with Cholbam (cholic acid capsules) — no efficacy data support concomitant use

Denial Risk

Not covered: combination with Cholbam

Requests for concomitant use of chenodiol products with Cholbam (cholic acid capsules) will be considered not medically necessary and are subject to denial.

Policy states there are no efficacy data to support concomitant use

Provider Actions & Documentation Requirements

Prior Authorization

Prior authorization required

Prior Authorization is recommended and approvals are provided for 1 year for approved indications; prescriber must be or consult a specialist appropriate to the condition.

Documentation Required

Diagnostic documentation for CTX

For CTX approvals, submit molecular genetic test demonstrating a pathogenic CYP27A1 variant or laboratory evidence of elevated serum cholestanol; include documentation of specialist prescribing or consultation.

Molecular genetic test demonstrating pathogenic CYP27A1 variant
Laboratory evidence of elevated serum cholestanol
Specialist prescribing or consultation documentation

Clinical Evidence

Evidence summary: Replacement therapy with chenodiol inhibits abnormal bile acid synthesis, reduces elevated plasma cholestanol concentrations, and eliminates bile alcohols in CTX. A CTX expert panel concluded that treatment with chenodiol is necessary to improve/stabilize prognosis in the majority of patients, regardless of age or symptoms (Stelten et al. 2021).

Background: Chenodiol products are naturally occurring bile acids used to dissolve radiolucent gallstones (Chenodal) and to replace deficient bile acids in cerebrotendinous xanthomatosis (CTX) (Ctexli). CTX is a rare lipid storage and bile acid synthesis disorder caused by pathogenic variants in the CYP27A1 gene leading to elevated serum cholestanol; chenodiol replacement therapy inhibits abnormal bile acid synthesis and is effective in reducing cholestanol and eliminating bile alcohols.

Definitions

TermCTX

DefinitionCerebrotendinous xanthomatosis, a lipid storage and bile acid synthesis disorder caused by pathogenic CYP27A1 variants.

Key featuresClinical manifestations include juvenile cataracts, tendon xanthomas, premature atherosclerosis, progressive neurologic disturbance (ataxia, seizures, psychiatric disorders, peripheral neuropathy).

Biochemical markersIncreased serum cholestanol and elevated urinary bile alcohols (e.g., 23S-pentol).

Treatment relevanceReplacement therapy with chenodiol inhibits abnormal bile acid synthesis and reduces elevated plasma cholestanol; expert panel concluded chenodiol is necessary to improve/stabilize prognosis in most patients.

Revision History

03/19/2025material_change

Policy name changed from 'Chenodal' to 'Chenodiol Products', criteria divided by specific agent, and Ctexli tablets were added to the policy; Ctexli: added condition of approval for CTX; Chenodal: removed CTX from other uses with supportive evidence.

08/28/2024annual_revision

Annual revision with no criteria changes.

03/04/2026annual_revisionLatest

Annual revision with no criteria changes; policy review date recorded as 03/04/2026.