CurrentMass General Brigham Health PlanPolicy N/A

Livmarli (maralixibat) — coverage criteria for cholestatic pruritus (PFIC, ALGS)

Defines prior authorization, quantity limits, specialty pharmacy dispensing, and medical necessity criteria for Livmarli (maralixibat) for treatment of cholestatic pruritus in PFIC and Alagille syndrome for Mass General Brigham Health Plan members.

Policy Summary

PayerMass General Brigham Health Plan

PolicyLivmarli (maralixibat) — coverage criteria for cholestatic pruritus (PFIC, ALGS)

Policy CodePolicy N/A

Change TypeMaterial clinical updates and additions

Effective DateN/A

Next Review DateN/A

Key ActionSubmit prior authorization with diagnosis, age, pruritus documentation, specialist involvement, required genetic testing, prior therapy history, and evidence of clinical response for reauthorization.

SourceLink

POLICY UPDATE CHANGES

Added approval criteria for supplemental indication of PFIC.

Updated criteria for Alagille syndrome to allow approval if the member has had genetic testing.

Added Livmarli oral tablet to the policy and specified quantity limits per formulation.

12 monthsApproval period

3 mL/daySolution limit

2/dayTablet limit (10/20 mg)

1/day

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Coverage and Medical Necessity Criteria

Initial Therapy - PFIC

Covered when ALL of the following are met:

PFIC initial criteria: Member has a diagnosis of progressive familial intrahepatic cholestasis (PFIC); member is 12 months of age or older; member has pruritus; requested medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a provider who specializes in PFIC; genetic testing does not indicate PFIC type 2 with ABCB11 variants encoding for nonfunction or absence of bile salt export pump (BSEP) protein; member has had an inadequate response, adverse reaction, or contraindication to at least ONE of the following: ursodiol (UDCA), antihistamine, rifampin, cholestyramine, sertraline, or naltrexone.

Initial Therapy - Alagille Syndrome (ALGS)

Covered when ALL of the following are met:

ALGS initial criteria: Member has a diagnosis of Alagille syndrome (ALGS) confirmed by genetic testing showing JAG1 or NOTCH2 mutation, bile duct paucity, or three of five major clinical features (e.g., cholestasis, cardiac defect, ophthalmologic abnormality, skeletal abnormality, characteristic facial features); member is 3 months of age or older; member has pruritus; requested medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a provider who specializes in treatment of Alagille syndrome; member has had an inadequate response, adverse reaction, or contraindication to at least ONE of the following: ursodiol (UDCA), antihistamine, rifampin, cholestyramine, sertraline, or naltrexone.

Documentation may include genetic testing or clinical features per policy.

Continuation Therapy

Requests for reauthorization will be approved when ALL of the following are met:

Continuation of therapy: Prescriber submits documentation of positive clinical response as evidenced by improvement in severity of pruritus; prior authorization conditions (diagnosis, specialist involvement, and required prior trial of alternatives) remain satisfied.

Approvals granted for 12 months.

Maralixibat (Livmarli) is not recommended for a subgroup of patients with Progressive Familial Intrahepatic Cholestasis (PFIC) type 2 whose genetic testing identifies ABCB11 variants that encode for non‑functional or absent bile salt export pump (BSEP) protein. This exclusion mirrors the drug labeling and the policy requirement that genetic testing not indicate PFIC type 2 with ABCB11 variants causing nonfunctional or absent BSEP protein.

Authorization will not be granted unless the member meets all specified criteria for the requested indication. Required elements include a qualifying diagnosis (PFIC or ALGS), applicable minimum age, documentation of pruritus, specialist prescribing or consultation as specified, any required genetic testing results (including exclusion of ABCB11 nonfunctional/absent BSEP variants for PFIC type 2), and evidence of prior trial and inadequate response, adverse reaction, or contraindication to at least one listed alternative therapy.

Quantity Limits, Approval Duration, and Coding

Quantity limits by formulationNDC | drug descriptors

Livmarli 9.5 mg/mL oral solution	Quantity limit: 3 mL per day
Livmarli 10 mg tablet	Quantity limit: 2 tablets per day
Livmarli 20 mg tablet	Quantity limit: 2 tablets per day
Livmarli 30 mg tablet	Quantity limit: 1 tablet per day

inv-07: Approval duration

Initial approval period12 months

Reauthorization period12 months

NotesInitial approvals and reauthorizations will be granted for 12 months as stated in policy limitations.

Prior Authorization, Documentation, and Prescriber Requirements

Prior Authorization

Prior Authorization Required

Prior authorization required. Transitional review may be performed for members new to the plan within the past 90 days who are currently receiving treatment with the requested medication (excluding when product obtained as samples or via manufacturer's patient assistance programs). Authorization may be granted only when member meets the specific indication criteria and other requirements listed below.

Transitional review: members new to plan within 90 days receiving treatment

Step Therapy

Step Therapy — Trial of Alternative Agent Required

Trial of at least one alternative agent is required unless contraindicated or clinically not appropriate. Prescriber must document an inadequate response, intolerance, or contraindication to one of the following prior to approval: ursodiol (UDCA), antihistamine, rifampin, cholestyramine, sertraline, or naltrexone.

Required trial: at least ONE of: ursodiol (UDCA), antihistamine, rifampin, cholestyramine, sertraline, naltrexone
Exception: documented contraindication or clinical reason why trial is inappropriate

Definitions and Indication Details

inv-13: PFIC

DefinitionProgressive familial intrahepatic cholestasis (PFIC) — a genetic cholestatic liver disease associated with pruritus; policy requires diagnosis of PFIC.

Minimum approvable ageAge ≥ 12 months

Required diagnostic elementsDiagnosis of PFIC; presence of pruritus; prescription by or in consultation with a hepatologist, gastroenterologist, or PFIC specialist; genetic testing must NOT indicate PFIC type 2 with ABCB11 variants encoding for nonfunction or absence of BSEP protein; prior inadequate response/adverse reaction/contraindication to at least one alternative (ursodiol, antihistamine, rifampin, cholestyramine, sertraline, or naltrexone).

inv-14: Alagille syndrome (ALGS)

DefinitionAlagille syndrome (ALGS) — a genetic disorder confirmed by genetic testing showing JAG1 or NOTCH2 mutation, or by bile duct paucity or three of five major clinical features (cholestasis, cardiac defect, skeletal abnormality, ophthalmologic abnormality, characteristic facies).

Drug Background and Mechanism

Maralixibat (Livmarli) is an ileal bile acid transporter (IBAT) inhibitor indicated for the treatment of cholestatic pruritus in: (1) patients with Progressive Familial Intrahepatic Cholestasis (PFIC) age 12 months and older, and (2) patients with Alagille syndrome (ALGS) age 3 months and older. The policy notes that maralixibat is not recommended for a subgroup of PFIC type 2 patients with ABCB11 variants resulting in non‑functional or absent BSEP protein.

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