CurrentMass General Brigham Health PlanPolicy N/A

Livmarli (maralixibat) prior authorization for cholestatic pruritus in ALGS and PFIC

Defines prior authorization requirements, eligibility criteria, quantity limits, and continuation guidance for Livmarli (maralixibat) under the pharmacy benefit for Mass General Brigham Health Plan commercial/exchange members.

Policy Summary

PayerMass General Brigham Health Plan

PolicyLivmarli (maralixibat) prior authorization for cholestatic pruritus in ALGS and PFIC

Policy CodePolicy N/A

Change TypeAdded tablet formulationrevised criteria and reauthorization interval

Effective DateJul 11, 2025

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation confirming diagnosis, required prior trial of alternatives, and genetic testing when applicable.

SourceLink

POLICY UPDATE CHANGES

Added Livmarli oral tablet formulation to the policy.

Initial approvals and reauthorizations updated to 12 months.

Approvals expanded to include PFIC supplemental indication and added criteria for genetic testing to exclude PFIC type 2 with specific ABCB11 variants.

12 monthsinitial and reauthorization approval duration

3 monthsminimum approvable age for Alagille syndrome

12 monthsminimum approvable age for PFIC

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.

Coverage Criteria for Livmarli (maralixibat)

Initial Therapy — PFIC

Covered when ALL of the following are met

PFIC initial criteria: 1. Member has a diagnosis of progressive familial intrahepatic cholestasis (PFIC). 2. Genetic testing does not indicate PFIC type 2 with ABCB11 variants encoding for nonfunction or absence of bile salt export pump (BSEP) protein. 3. Member is 12 months of age or older. 4. Requested medication is being prescribed by or in consultation with a hepatologist, gastroenterologist, or a provider who specializes in PFIC. 5. Member has pruritus. 6. Member has had an inadequate response, adverse reaction, or contraindication to at least ONE of the following: a. Ursodiol (UDCA); b. Antihistamine; c. Rifampin; d. Cholestyramine; e. Sertraline; f. Naltrexone.

Initial Therapy — Alagille syndrome (ALGS)

Covered when ALL of the following are met

ALGS initial criteria: 1. Diagnosis of Alagille syndrome (ALGS) confirmed by ONE of the following: a. Documentation of genetic testing confirming mutations of the JAG1 or NOTCH2 genes; b. Bile duct paucity; c. THREE of the five major clinical features of ALGS: i. Cholestasis; ii. Cardiac defect (e.g., peripheral pulmonary artery stenosis); iii. Skeletal abnormality (e.g., butterfly vertebrae); iv. Ophthalmologic abnormality (e.g., posterior embryotoxon); v. Characteristic facial features (e.g., triangular face, broad forehead, pointed chin, bulbous nasal tip, deeply set eyes, hypertelorism). 2. Member is 3 months of age or older. 3. Requested medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or provider who specializes in treatment of Alagille syndrome. 4. Member has pruritus. 5. Member has had an inadequate response, adverse reaction, or contraindication to at least ONE of the following: a. Ursodiol (UDCA); b. Antihistamine; c. Rifampin; d. Cholestyramine; e. Sertraline; f. Naltrexone.

Continuation of Therapy

Reauthorization is approved when ALL of the following are met

Continuation criteria: 1. Prescriber submits documentation of positive clinical response as evidenced by improvement in severity of pruritus. 2. (Operational) Reauthorizations and initial approvals will be granted for up to 12 months; ensure quantity limits by formulation are adhered to per policy.

Livmarli is not recommended for a subgroup of PFIC type 2 patients who have ABCB11 variants that encode nonfunctional or absent bile salt export pump (BSEP) protein. For PFIC initial therapy requests, the policy requires genetic testing that specifically excludes these ABCB11 variants as part of medical necessity determination. Documentation should clearly state genetic test results demonstrating the absence of ABCB11 variants that would produce nonfunctional or absent BSEP before approval is granted. Requests that do not include genetic testing results or that identify qualifying ABCB11 variants consistent with nonfunctional/absent BSEP should be denied as not appropriate for maralixibat therapy.

Provider Requirements and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required for coverage of Livmarli (maralixibat). Submit prior authorization requests to the pharmacy benefit contact listed on the plan header. Authorization may be reviewed for members new to the plan within the past 90 days who are currently receiving treatment with the requested medication (excluding samples or patient assistance program supplies).

Pharmacy benefit prior auth phone: 800-711-4555
Pharmacy benefit prior auth fax: 844-403-1029

Step Therapy

Required Trial of Alternatives

Member must have had an inadequate response, adverse reaction, or contraindication to at least one of the specified alternative therapies before initial authorization for the requested indication will be granted.

PFIC alternatives (at least one): Ursodiol (UDCA); Antihistamine; Rifampin; Cholestyramine; Sertraline; Naltrexone
ALGS alternatives (at least one): Ursodiol (UDCA); Antihistamine; Rifampin; Cholestyramine; Sertraline; Naltrexone

Key Definitions

PFIC type 2 with ABCB11 variants encoding nonfunctional or absent BSEP protein

DefinitionPFIC type 2 with ABCB11 variants encoding nonfunctional or absent BSEP protein refers to a subgroup of progressive familial intrahepatic cholestasis in which ABCB11 gene variants result in non-functional or complete absence of the bile salt export pump (BSEP) protein.

Policy implicationLivmarli is not recommended for members whose genetic testing indicates PFIC type 2 with ABCB11 variants that encode nonfunctional or absent BSEP protein; genetic testing must not indicate these variants for approval.

Minimum approvable ageMember must be 12 months of age or older for PFIC treatment with Livmarli.

Required documentationPrescriber must provide genetic testing results that exclude ABCB11 variants encoding nonfunctional or absent BSEP protein as part of the prior authorization documentation.

Alagille syndrome (ALGS) — diagnostic criteria

Background

Livmarli (maralixibat) is an ileal bile acid transporter (IBAT) inhibitor approved for the treatment of cholestatic pruritus in patients with Alagille syndrome (ALGS) aged ≥3 months and in patients with progressive familial intrahepatic cholestasis (PFIC) aged ≥12 months. The policy notes that Livmarli is not recommended for a subgroup of PFIC type 2 patients with specific ABCB11 variants that result in nonfunctional or absent BSEP protein.