Linerixibat Long-term Safety, and Tolerability Study

Inclusion Criteria:

• Male and female participants must be 18 to 80 years of age inclusive, at the time ofsigning the informed consent in the participant's parent trial BAT117213, GLIMMER orGLISTEN.

• Participants with a diagnosis of PBC and a history of associated pruritus asevidenced by randomization into a prior eligible linerixibat clinical trial (BAT117213, GLIMMER or GLISTEN).

• Participants must have completed the main treatment period in a prior eligiblelinerixibat clinical trial (BAT117213, GLIMMER or GLISTEN).

• A female participant is eligible to participate if she is not pregnant orbreastfeeding, and at least one of the following conditions applies: a) is a womanof non-childbearing potential (WONCBP) or b) is a woman of childbearing potential (WOCBP) and using an acceptable contraceptive method.

• Capable of giving signed informed consent.

Exclusion Criteria:

• Screening total bilirubin >2x upper limit of normal (ULN).

• Screening alanine aminotransferase (ALT) or aspartate aminotransferase (AST) >6xULN.

• Screening estimated glomerular filtration rate (eGFR) <30 milliliters per minute per 1.73 square meter (mL/min/1.73m^2) based on the Chronic Kidney Disease EpidemiologyCollaboration (CKD-EPI) equation.

• Presence of hepatic decompensation (e.g., variceal bleeds, encephalopathy orascites).

• Presence of actively replicating viral hepatitis B or C (Viral Hepatitis B [HBV],viral Hepatitis C [HCV]) infection), primary sclerosing cholangitis (PSC), alcoholicliver disease and/or confirmed hepatocellular carcinoma or biliary cancer.

• Current clinically significant diarrhea in the Investigator's medical opinion.

• Current symptomatic cholelithiasis or cholecystitis.

• Current diagnosis or previous diagnosis of colorectal cancer.

• Any current malignancies (including hematologic and solid malignancies).

• History of bariatric surgery with ileal bypass at any time, or any bariatric surgeryperformed in the past 3 years.

• Use of Obeticholic acid: within 8 weeks prior to the date of the screening visit andmay not restart until after the end of the study or early study withdrawal.

• Administration of any other ileal bile acid transporter (IBAT) inhibitor in the 1month prior to screening until after the end of the study or early study withdrawal.

• QT interval corrected (QTc) >480 millisecond (msec) at screening (12-lead ECG)

• Participants with moderate (or greater) alcohol consumption defined as more than onestandard drink per day for women and two drinks per day for men.