ModifiedmhnPolicy CP.PHAR.543

Maralixibat (Livmarli) coverage for cholestatic pruritus

Defines medical necessity, prior authorization, dosing, and coverage criteria for maralixibat (Livmarli) for treatment of cholestatic pruritus in Alagille syndrome and progressive familial intrahepatic cholestasis for applicable Centene lines of business.

Policy Summary

Payermhn

PolicyMaralixibat (Livmarli) coverage for cholestatic pruritus

Policy CodePolicy CP.PHAR.543

Change TypeMaterial coverage and dosing updates

Effective DateSep 29, 2021

Next Review Date

Key ActionSubmit prior authorization with chart notes, labs, and current body weight (kg) demonstrating member meets indication-specific criteria and dosing requirements.

SourceLink

POLICY UPDATE CHANGES

Added tablet formulations (10 mg, 15 mg, 20 mg, 30 mg) for ALGS and PFIC and updated criteria to address requests for tablets including weight ≥ 25 kg.

Updated dosing and strengths: added 19 mg/mL oral solution for PFIC and 9.5 mg/mL for ALGS; aligned maximum dosing with prescribing information.

Added exclusions for portal hypertension and history of hepatic decompensation for both PFIC and ALGS in initial criteria.

Added exclusion for concurrent use with other IBAT inhibitors for ALGS initial/continued therapy and PFIC continued therapy.

2FDA-indicated diagnoses

≥3 mo / ≥12 moage minimums (ALGS/PFIC)

2oral solution strengths

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Coverage Criteria for Maralixibat (Livmarli)

Initial Approval — Alagille Syndrome

Covered when ALL of the following are met

ALGS Initial Criteria: 1) Diagnosis of ALGS-associated pruritus confirmed by one of the following: a. Genetic confirmation with presence of a mutation in JAG1 or NOTCH2; OR b. Clinical confirmation with both: i. bile duct paucity on liver biopsy; AND ii. criteria meeting ≥3 of the 5 classic major criteria (see Appendix D). 2) Prescribed by or in consultation with a hepatologist or gastroenterologist. 3) Age ≥ 3 months and ≤ 18 years at therapy initiation. 4) Pruritus requiring at least moderate scratching (e.g., ≥2 on 0–4 ItchRO). 5) Evidence of cholestasis meeting at least one of: a. total serum bile acid >3× ULN for age; b. conjugated bilirubin >1 mg/dL; c. unexplained fat‑soluble vitamin deficiency; d. GGT >3× ULN for age; e. intractable pruritus attributable to liver disease. 6) Member does not have portal hypertension or history of hepatic decompensation. 7) Failure of ursodeoxycholic acid unless contraindicated or intolerant (note: Illinois HIM exception for step therapy applies per IL HB 5395). 8) Failure of symptomatic agents for pruritus (e.g., antihistamines, rifampin, cholestyramine) unless contraindicated or intolerant. 9) Not prescribed concurrently with other IBAT inhibitors (e.g., Bylvay). 10) Documentation of member's current body weight in kilograms. 11) If request is for oral solution, request must be for 9.5 mg/mL strength. 12) If request is for tablets, documentation of member's current body weight ≥25 kg. 13) Dose does not exceed ALGS limits: for oral solution 380 mcg/kg/day (maximum 28.5 mg/day); for tablets 30 mg/day (1 tablet per day).

Policy lists exact diagnostic, prior-treatment, formulation, weight and dosing limits.

Supporting documentation (one required): Provider must submit chart notes, relevant laboratory results (including sBA, bilirubin, GGT, vitamin levels), weight in kg, and documentation of prior therapies and response/intolerance.

See required documentation/authorization sections.

Initial Approval — Progressive Familial Intrahepatic Cholestasis (PFIC)

Covered when ALL of the following are met

PFIC Initial Criteria: 1) Diagnosis of genetically confirmed PFIC with both: a. moderate to severe pruritus (e.g., ≥1.5 on 0–4 scale); AND b. serum bile acid (sBA) >3× ULN for age. 2) Prescribed by or in consultation with a hepatologist or gastroenterologist. 3) Age ≥ 12 months at therapy initiation. 4) For PFIC type 2, member must NOT have ABCB11 gene variants resulting in nonfunctional or absent BSEP protein. 5) Member does not have portal hypertension or history of hepatic decompensation. 6) Failure of ursodeoxycholic acid unless contraindicated or intolerant (note: Illinois HIM exception per IL HB 5395). 7) Failure of symptomatic agents for pruritus (e.g., antihistamines, rifampin, cholestyramine) unless contraindicated or intolerant. 8) Not prescribed concurrently with other IBAT inhibitors (e.g., Bylvay). 9) Documentation of member's current body weight in kilograms. 10) If request is for oral solution, request must be for 19 mg/mL strength. 11) If request is for tablets, documentation of member's current body weight ≥25 kg. 12) Dose does not exceed PFIC limits: for oral solution 1,140 mcg/kg/day (maximum 38 mg/day); for tablets 40 mg/day (2 tablets per day).

Includes genetic exclusion for PFIC2 BSEP deficiency and formulation/weight limits.

Supporting documentation (one required): Provider must submit chart notes, labs (sBA and other relevant tests), weight in kg, and documentation of prior therapies and response/intolerance.

See required documentation/authorization sections.

Continuation/Renewal Criteria — ALGS and PFIC

Covered when ALL of the following are met

Continuation Criteria: 1) Member is currently receiving medication via the Centene benefit or previously met initial approval criteria, or continuity of care provisions apply. 2) Member is responding positively to therapy: for ALGS, documented improvement in pruritus; for PFIC, documented improvement in pruritus and/or reduction in sBA from baseline. 3) Livmarli is not prescribed concurrently with other IBAT inhibitors. 4) Documentation of member's current body weight in kilograms. 5) If request is for oral solution, correct concentration must be requested (ALGS: 9.5 mg/mL; PFIC: 19 mg/mL). 6) If request is for tablets, documentation of member's current body weight ≥25 kg. 7) If request is for a dose increase, the new dose does not exceed the indication‑specific maximums (ALGS oral solution 380 mcg/kg/day up to 28.5 mg/day; ALGS tablets 30 mg/day; PFIC oral solution 1,140 mcg/kg/day up to 38 mg/day; PFIC tablets 40 mg/day).

Renewal requires objective clinical response and compliance with formulation/weight/dosing limits.

Dosing and formulation criteria (ALGS and PFIC)

Covered when dosing, formulation, and eligibility criteria per FDA labeling and policy are met

ALGS dosing: Oral solution: start 190 mcg/kg PO once daily for days 1–7, then increase to 380 mcg/kg PO once daily as tolerated; maximum 380 mcg/kg/day (maximum 28.5 mg/day). If request is for oral solution, the requested concentration must be 9.5 mg/mL. Tablet formulations are available (10, 15, 20, 30 mg strengths); tablet use requires documentation of member's current body weight ≥25 kg and tablet dosing must not exceed 30 mg/day (1 tablet/day).

Dosing regimens and available strengths aligned with prescribing information and policy changes.

PFIC dosing: Oral solution: start 285 mcg/kg PO once daily then titrate to 285 mcg/kg PO twice daily, then 428 mcg/kg PO twice daily, and up to 570 mcg/kg PO twice daily as tolerated; maximum 1,140 mcg/kg/day (maximum 38 mg/day). If request is for oral solution, the requested concentration must be 19 mg/mL. Tablet formulations are available (10, 15, 20, 30 mg strengths); tablet use requires documentation of member's current body weight ≥25 kg and tablet dosing must not exceed 40 mg/day (2 tablets/day).

PFIC titration schedule and maximums per prescribing information; policy added 19 mg/mL solution and tablet options.

Requests for maralixibat for indications that are not FDA‑approved and not specifically addressed in this policy will not be authorized unless the provider submits sufficient documentation of efficacy and safety consistent with the plan’s off‑label use policies (e.g., CP.CPA.09 for commercial, HIM.PA.154 for marketplace, CP.PMN.53 for Medicaid) or there is other applicable evidence of coverage supporting the use.

The policy excludes members who have portal hypertension or a history of hepatic decompensation from initial approval for both Alagille syndrome and PFIC. Additionally, for ALGS initial/continued therapy and for PFIC continued therapy, the policy excludes concurrent use with other ileal bile acid transporter (IBAT) inhibitors. These exclusions were added to align with comparator criteria and were incorporated in the 3Q 2024 and 3Q 2025 updates.

The policy specifically notes that maralixibat is unlikely to be effective in PFIC type 2 patients who have ABCB11 variants that result in absent or nonfunctional BSEP protein, since the drug acts on bile acid transport and requires the presence of the bile salt export pump to exert its intended effect.

Policy history documents multiple revisions: a 3Q 2023 update adding the ItchRO appendix and pediatric age changes; a 3Q 2024 annual review adding exclusions for portal hypertension and prior hepatic decompensation; and a 3Q 2025 update that added tablet formulations and weight‑based tablet criteria (documentation of current body weight ≥ 25 kg), clarified solution strengths, added the concurrent‑IBAT exclusion for certain uses, and implemented a step‑therapy bypass for Illinois HIM per IL HB 5395.

Coding and Clinical Thresholds

No explicit CPT/HCPCS/ICD-10 codes present in this document sectionmixed

No codes listed

Serum bile acid (sBA) threshold — definition

sBA thresholdSerum bile acid (sBA) > 3 times the upper limit of normal (ULN) for age

Clinical useRequired as evidence of cholestasis for ALGS initial approval (one of criteria a–e) and as a specific PFIC entry requirement

Measurement contextCompared to age-specific ULN as reported in laboratory results

Pruritus severity thresholds

ALGS pruritus thresholdPruritus requiring at least moderate scratching (≥2 on 0–4 ItchRO) for ALGS initial criteria

PFIC pruritus thresholdModerate to severe pruritus (≥1.5 on 0–4 scale) required for PFIC initial criteria

Provider Requirements, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required. Provider must demonstrate the member meets the initial approval criteria for the relevant indication and submit supporting documentation (office chart notes, lab results, genetic testing, dosing calculations, and other clinical information).

Prior authorization required for Livmarli (maralixibat) per CP.PHAR.543
Include documentation supporting ALL criteria met (diagnosis, symptom severity, prior therapies, weight in kg, dosing)

Documentation Required

Formulation- and Strength-Specific PA Requirement

Requests must specify the exact formulation and strength being requested. For oral solution, indicate the strength (9.5 mg/mL for Alagille Syndrome; 19 mg/mL for PFIC where applicable). For tablets, indicate tablet strength and ensure tablet eligibility criteria are met.

Oral solution strengths: 9.5 mg/mL (ALGS) or 19 mg/mL (PFIC) — do not substitute strengths

Background

Maralixibat (Livmarli) is an oral ileal bile acid transporter (IBAT) inhibitor indicated for cholestatic pruritus associated with Alagille syndrome (approved for use in patients aged ≥ 3 months) and for progressive familial intrahepatic cholestasis (PFIC) (approved for use in patients aged ≥ 12 months per the policy updates). It reduces reabsorption of bile acids in the ileum to lower serum bile acids and improve pruritus. The product is supplied as oral solution in two strengths (commonly referenced as 9.5 mg/mL for ALGS and 19 mg/mL for PFIC) and is also available in tablet formulations; dosing regimens and maximum daily doses are specified by indication and were aligned with the prescribing information in recent updates.

Definitions and Clinical Measures

sBA threshold definition

sBA threshold definitionserum bile acid (sBA) > 3 times the upper limit of normal (ULN) for age

Role in criteriaUsed as evidence of cholestasis for ALGS (one of multiple options) and as a required PFIC entry criterion

Reference locationDefined in Initial Approval Criteria and Appendix A abbreviations

ItchRO — caregiver-observed pruritus scale

ItchRO descriptionCaregiver‑observed 0–4 Itch Reported Outcome (ItchRO) scale measuring scratching twice daily (morning and evening)

Scoring5‑point scale: 0 none; 1 mild; 2 moderate; 3 severe; 4 very severe