CurrentCignaPolicy N/A

Chenodiol Products Prior Authorization Policy

Prior authorization requirements for prescription coverage of chenodiol products (Chenodal and Ctexli) for Cigna-administered health benefit plans; defines coverage criteria, prescribing specialist requirements, and non-covered uses.

Policy Summary

PayerCigna

PolicyChenodiol Products Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateMar 19, 2025

Key ActionSubmit prior authorization and documentation showing diagnostic confirmation (for CTX) or trial/current use of an ursodiol product (for gallstones) and that the medication is prescribed by or in consultation with an appropriate specialist.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2Products covered

1 yrTypical approval duration

1CTX diagnostic + specialist required

1Ursodiol trial required for gallstones

Coverage Criteria

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inv-02: Initial Therapy — Ctexli for Cerebrotendinous Xanthomatosis

Covered when ALL of the following are met:

Ctexli - CTX diagnostic and specialist criteria: Approve for 1 year if the patient meets BOTH of the following: A) The diagnosis is established by ONE of the following: i) molecular genetic test demonstrating a pathogenic CYP27A1 variant; OR ii) laboratory test demonstrating elevated serum cholestanol levels; AND B) The 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.

Diagnostic confirmation plus specialist involvement required.

Combination therapy of chenodiol products with Cholbam (cholic acid capsules) is not supported by the available evidence. The policy lists concomitant use of chenodiol and cholic acid as lacking efficacy data and classifies this combination as experimental/investigational, and therefore it is not an accepted coverage approach.

Requests for Chenodal or Ctexli for any indication other than the specific FDA‑approved uses described in this policy are considered experimental, investigational, or unproven. This explicitly includes combination therapy with Cholbam (cholic acid capsules) and may be denied due to lack of supporting efficacy data.

Initial Therapy Criteria (by product)

inv-13: Chenodal — Initial Therapy Criteria (Travere) detailed criteria block

Covered when ONE of the following is met:

Chenodal - Initial Therapy (Travere): Approve for 1 year if the patient meets ONE of the following: A) Patient has tried an ursodiol product; OR B) Patient is currently receiving an ursodiol product.

Medication must be prescribed by or in consultation with a physician who specializes in the condition being treated.

inv-14: Ctexli — Initial Therapy Criteria (Mirum) detailed criteria block

Covered when ALL of the following are met:

Ctexli - Initial Therapy (Mirum): Approve for 1 year if the patient meets BOTH of the following: A) Diagnosis of CTX is established by ONE of the following: i) molecular genetic test demonstrating a pathogenic CYP27A1 variant; OR ii) laboratory test demonstrating elevated serum cholestanol levels; AND B) Medication is prescribed by or in consultation with a geneticist, neurologist, ophthalmologist, metabolic specialist who treats patients with cerebrotendinous xanthomatosis or another specialist who focuses in the treatment of cerebrotendinous xanthomatosis.

Diagnostic confirmation plus specialist prescribing/consultation required.

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization required — approvals provided for 1 year when criteria met.

Note

Ursodiol Trial Requirement

For Chenodal (gallstones), patient must have tried or be currently receiving an ursodiol product. Documentation should include evidence of prior or current ursodiol therapy (medication history, prescription records, or clinical notes).

Applies to FDA-approved gallstone indication: patient has tried an ursodiol product OR is currently receiving an ursodiol product.
Document: medication history, prescription records, or clinical progress notes showing ursodiol trial/therapy.

Documentation Required

Documentation Required for CTX

Documentation required for cerebrotendinous xanthomatosis (CTX) — confirmatory diagnostic evidence must be provided. Acceptable documentation includes molecular genetic testing showing a pathogenic CYP27A1 variant OR laboratory evidence of elevated serum cholestanol, plus documentation that the medication is prescribed by or in consultation with an appropriate specialist (geneticist, neurologist, ophthalmologist, metabolic specialist, or clinician experienced in CTX).

Acceptable diagnostic evidence: molecular genetic test demonstrating pathogenic CYP27A1 variant OR lab test showing elevated serum cholestanol.
Specialist prescriber or documented consultation: geneticist, neurologist, ophthalmologist, metabolic specialist, or other specialist who treats CTX.

Documentation Required

Documentation Required for Gallstones

Documentation required for gallstones — include evidence of an ursodiol trial or current ursodiol therapy when requesting chenodiol for gallstones.

Provide medication history, prescription records, or clinical notes documenting ursodiol use.
If patient is currently receiving ursodiol, include recent prescriptions or administration records.

Denial Risk

Non‑Covered Uses

Non-covered uses — requests for chenodiol products for any use other than the listed FDA-approved indications (gallstones and cerebrotendinous xanthomatosis) are not covered.

Step	Requirement	Coverage
1	Patient has tried an ursodiol product	Approval for 1 year if requirement met
1	Patient is currently receiving an ursodiol product	Approval for 1 year if requirement met

Background

Chenodiol products are naturally occurring primary bile acids used in two clinical contexts: to promote dissolution of radiolucent (cholesterol-predominant) gallstones in patients for whom cholecystectomy is high risk, and to replace deficient bile acids in cerebrotendinous xanthomatosis (CTX). In CTX, pathogenic variants in CYP27A1 impair primary bile acid synthesis, leading to accumulation of cholestanol and other bile alcohols; chenodiol replacement helps inhibit abnormal bile acid synthesis. Treatment for gallstones with bile acids (chenodiol or ursodiol) typically requires prolonged therapy over months to years to achieve dissolution.

Definitions

Cerebrotendinous xanthomatosis (CTX)

DefinitionCerebrotendinous xanthomatosis (CTX) is a lipid storage and bile acid synthesis disorder characterized by juvenile cataracts, tendon xanthomas, progressive neurologic disturbance, elevated serum cholestanol, and elevated urinary bile alcohols.

Genetic causeCaused by pathogenic variants in the CYP27A1 gene leading to sterol 27‑hydroxylase (27‑hydroxylase) deficiency and reduced primary bile acid synthesis.

PathophysiologyDeficient 27‑hydroxylase reduces conversion of cholesterol to primary bile acids (cholic and chenodeoxycholic acids), causing failed feedback inhibition of cholesterol production and accumulation of cholestanol and bile alcohols.

Diagnostic evidenceDiagnosis is established using biochemical tests (elevated serum cholestanol) and/or molecular genetic testing identifying pathogenic CYP27A1 variants.

Treatment rationaleChenodiol replacement therapy inhibits abnormal bile acid synthesis, reduces plasma cholestanol, and eliminates bile alcohols; expert panel concluded chenodiol is necessary to improve/stabilize prognosis in most patients.

Radiolucent gallstones

DefinitionRadiolucent gallstones are cholesterol‑predominant gallstones that are amenable to dissolution with bile acid therapy, appearing radiolucent on imaging.

Usual treatment courseBile acid therapy (chenodeoxycholic acid/chenodiol or ursodeoxycholic acid/ursodiol) promotes gradual dissolution of radiolucent gallstones over ~6 months to 2 years.

Therapeutic agentsTwo naturally occurring bile acids used: ursodiol (ursodeoxycholic acid) and chenodiol (chenodeoxycholic acid, Chenodal).

Place in careCholecystectomy is the most widely used treatment for symptomatic gallstones; bile acid therapy is an alternative for radiolucent stones in patients where surgery is high risk.