CurrentCignaPolicy IP0363

Bylvay

Defines Cigna prior authorization and coverage criteria for Bylvay (odevixibat) for treatment of pruritus in progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (ALGS) for applicable benefit plans.

Policy Summary

PayerCigna

PolicyBylvay

Policy CodePolicy IP0363

Change TypeAnnual revisionmaterial changes

Effective Date10/1/2025

Next Review Date

Key ActionObtain prior authorization; initial approvals for new patients are for 6 months (continuing patients: 1 year).

SourceLink

POLICY UPDATE CHANGES

Policy name updated from 'Odevixibat' to 'Bylvay' and added 'Patient is Currently Receiving Bylvay' criteria for PFIC and ALGS.

Updated requirement wording to 'Patient has moderate-to-severe pruritus, according to the prescriber'.

Fenofibrate was added to the examples of systemic medications that should be tried prior to approval.

2FDA‑approved indications covered (PFIC, Alagille syndrome)

6 moInitial approval duration for new patients

1 yrApproval duration for patients currently receiving Bylvay

≥2Systemic medications required prior to initiation

Coverage Criteria for Bylvay (odevixibat)

Policy Summary

PayerCigna

PolicyBylvay

Policy CodePolicy IP0363

Change TypeAnnual revisionmaterial changes

Effective Date10/1/2025

Next Review Date

Key ActionObtain prior authorization; initial approvals for new patients are for 6 months (continuing patients: 1 year).

SourceLink

On This Page

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Coverage criteria for Bylvay (PFIC and ALGS)

Covered when ALL of the following are met for each indication.

Indication

PFIC - Initial Therapy
- Age: Patient is ≥ 3 months of age
- Symptom severity: Patient has moderate-to-severe pruritus, according to the prescriber
- Genetic confirmation: Diagnosis of PFIC confirmed by genetic testing demonstrating a pathogenic gene variant affiliated with PFIC (examples: ATP8B1, ABCB11, ABCB4, TJP2, NR1H4, MYO5B) [documentation required]
- Laboratory: Serum bile acid concentration above the upper limit of the normal reference range for the reporting laboratory
- Prior therapies: Patient has tried at least two systemic medications for PFIC (examples: fenofibrate, cholestyramine, naltrexone, rifampicin, sertraline, ursodeoxycholic acid [ursodiol]) unless contraindicated
- No advanced liver disease: Patient does NOT have cirrhosis, portal hypertension, or history of a hepatic decompensation event (examples: variceal hemorrhage, ascites, hepatic encephalopathy)
- Specialist prescriber: Medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in PFIC
PFIC - Current Therapy
- No advanced liver disease: Patient does NOT have cirrhosis, portal hypertension, or history of a hepatic decompensation event
- Response: Patient had response to therapy, as determined by the prescriber (examples: decrease in serum bile acids and decrease in pruritus)
- Specialist prescriber: Medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in PFIC
ALGS - Initial Therapy
- Age: Patient is ≥ 12 months of age
- Symptom severity: Patient has moderate-to-severe pruritus, according to the prescriber
- Genetic confirmation: Diagnosis of ALGS confirmed by genetic testing demonstrating a JAG1 or NOTCH2 deletion or pathogenic variant [documentation required]
- Laboratory: Serum bile acid concentration above the upper limit of the normal reference range for the reporting laboratory
- Prior therapies: Patient has tried at least two systemic medications for ALGS (examples: fenofibrate, cholestyramine, naltrexone, rifampicin, sertraline, ursodeoxycholic acid [ursodiol]) unless contraindicated
ALGS - Current Therapy
- No advanced liver disease: Patient does NOT have cirrhosis, portal hypertension, or history of a hepatic decompensation event
- Response: Patient had response to therapy, as determined by the prescriber (examples: decrease in serum bile acids and decrease in pruritus)
- Specialist prescriber: Medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in ALGS

Coverage stance: Bylvay for any other use is considered not medically necessary.

Bylvay for any other use is considered not medically necessary.

Use of Bylvay for indications other than progressive familial intrahepatic cholestasis (PFIC) or Alagille syndrome (ALGS) is considered not medically necessary.

Provider Requirements, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for Bylvay. Approvals are time-limited: initial therapy approvals for new patients are for 6 months; continuation/currently receiving approvals are for 1 year. Because of the specialized skills required for evaluation and diagnosis and monitoring, Bylvay must be prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in the condition being treated. Documentation is required where noted in the criteria and may include chart notes, laboratory tests, genetic testing results, prior therapy documentation, and claims records.

Prior authorization required for Bylvay
Initial therapy approvals (new patients): 6 months
Continuation/Currently receiving approvals: 1 year
Medication must be prescribed by or in consultation with a hepatologist, gastroenterologist, or specialist

Step Therapy

Step Therapy / Prior Trials

For initial therapy, patient must have tried at least two systemic medications for the relevant diagnosis (unless contraindicated). Examples of systemic medications include fenofibrate, cholestyramine, naltrexone, rifampicin, sertraline, and ursodeoxycholic acid (ursodiol). For patients currently receiving Bylvay, continuation approvals require documentation of clinical response as determined by the prescriber (for example, decrease in serum bile acids and/or decrease in pruritus).

Initial therapy: trial of ≥ 2 systemic medications unless contraindicated (examples: fenofibrate; cholestyramine; naltrexone; rifampicin; sertraline; ursodeoxycholic acid/ursodiol)
Patient is Currently Receiving Bylvay: documentation of response to therapy required for 1-year approval (examples: decreased serum bile acids, decreased pruritus)

Documentation Required

Documentation Requirements

Documentation must be submitted as indicated in the coverage criteria. Acceptable documentation includes, but is not limited to, chart notes, laboratory test results (including serum bile acid concentrations with reference ranges), genetic testing reports confirming pathogenic variants or deletions as specified for the diagnosis, and records demonstrating prior trials of systemic medications (including dates, doses, and reasons for discontinuation if applicable). Documentation should clearly support age criteria, diagnosis confirmation, absence of exclusionary conditions (cirrhosis, portal hypertension, history of hepatic decompensation), prior therapy trials or contraindications, and clinical response for continuation requests.

Chart notes supporting symptoms (e.g., moderate-to-severe pruritus) and prescriber assessment
Genetic testing report confirming pathogenic variant or deletion (e.g., PFIC genes: ATP8B1, ABCB11, ABCB4, TJP2, NR1H4, MYO5B; Alagille: JAG1 or NOTCH2)
Laboratory results showing serum bile acid concentration with lab reference range
Documentation of trials of ≥ 2 systemic medications (dates, doses, outcome, or contraindication/intolerance)
For continuation: documentation of response to therapy (e.g., decreased serum bile acids, decreased pruritus)
Evidence that prescribing/consulting clinician is a hepatologist, gastroenterologist, or relevant specialist

Denial Risk

Denial Triggers

Denial is likely if documentation is missing or does not meet coverage criteria, including: lack of a covered diagnosis, no genetic confirmation when required, absence of serum bile acid results above the lab reference range, failure to document trials of at least two systemic medications (or valid contraindication), presence of exclusionary conditions (cirrhosis, portal hypertension, history of hepatic decompensation), or if Bylvay is requested for indications considered not medically necessary.

Missing required genetic testing documentation when indicated
No laboratory evidence of elevated serum bile acids
No documentation of ≥ 2 prior systemic medication trials (or documented contraindication/intolerance)
Presence of exclusionary conditions: cirrhosis, portal hypertension, or history of hepatic decompensation
Requested use not matching FDA-approved indications

Key Definitions

IBAT inhibitor / Bylvay

Drug name (generic/brand)Odevixibat (Bylvay)

Mechanism of actionIleal bile acid transporter (IBAT) inhibitor

FDA‑approved usesTreatment of pruritus in patients ≥ 3 months of age with progressive familial intrahepatic cholestasis (PFIC); treatment of cholestatic pruritus in patients > 12 months of age with Alagille syndrome (ALGS)

Clinical effectMay reduce serum bile acids by inhibiting bile salt reuptake in the ileum

Commonly referenced comparators/adjunctsCholestyramine, rifampicin, ursodeoxycholic acid (ursodiol); fenofibrate, naltrexone, sertraline noted in guidelines

Progressive Familial Intrahepatic Cholestasis (PFIC)

DefinitionProgressive familial intrahepatic cholestasis (PFIC) is a group of rare, autosomal recessive liver diseases caused by pathogenic variants affecting bile acid transport (examples: ATP8B1, ABCB11, ABCB4, TJP2, NR1H4, MYO5B)

Typical clinical featuresCholestasis, jaundice, and pruritus; can progress to liver fibrosis, cirrhosis, or end‑stage liver disease

Onset and prognosisOften presents in infancy to adolescence and can lead to early death without treatment

Role of geneticsCaused by mutations in genes affecting bile acid transport, requiring genetic confirmation for diagnosis in policy criteria

Alagille syndrome (ALGS)

DefinitionAlagille syndrome (ALGS) is a rare genetic liver disease caused by deletion or pathogenic variants in JAG1 or NOTCH2, associated with cholestasis and pruritus

Typical clinical featuresCholestasis, jaundice, and pruritus; may progress to fibrosis, cirrhosis, or end‑stage liver disease

Age consideration for Bylvay useBylvay is indicated for cholestatic pruritus in ALGS in patients > 12 months of age

OpenPayer

Bylvay

Coverage Criteria for Bylvay (odevixibat)

Provider Requirements, Prior Authorization, and Documentation

Key Definitions

Background

Policy Revision History