Inclusion Criteria:  Males and females between 18 and 80 years of age (inclusive) who are able to comprehend instructions and follow the study procedures and are willing to sign an Informed Consent Form (ICF) Females of childbearing potential who are sexually active with a non-sterile male partner (sterile male partners are defined as men vasectomized since at least 6 months) must be willing to use the contraceptive methods throughout the study and for 30 days after study drug administration. For at least 90 days after study drug administration, non-vasectomized males must not donate sperm, be willing to use contraception with childbearing potential partners and any male subject with a pregnant partner must use a condom. Willing to abstain from consuming grapefruit, pomelo, star fruit, or Seville orange containing products from 7 days prior to dose of study medication through day of discharge. Confirmed diagnosis of PBC with evidence of cirrhosis and Child-Pugh classification of CP-A, CP-B or CP-C  Screening laboratory parameters:  ALP, ALT and AST < 10 × ULN Total bilirubin ≤ 5 × ULN Ursodeoxycholic acid (UDCA) for a minimum of 12 weeks of treatment prior to Day 1 At screening confirmed diagnosis of PBC MELD-Na scores of 6 to 24  Exclusion Criteria:  Clinically significant or history of acute or chronic liver disease of an etiology other than PBC Patients with a diagnosis of overlapping PBC and autoimmune hepatitis History, evidence, or high suspicion of hepatobiliary malignancy based on imaging, screening laboratory values, and/or clinical symptoms. Presumptive or diagnosed infection that requires systemic therapy within 12 weeks of Screening and through Day 1 Female subjects who are pregnant or nursing Screening ECG that demonstrates a QT interval ≥ 500 msec, or any other significant ECG finding with clinically significant abnormalities as determined by the Investigator Positive for HBsAg, HCV RNA, or anti HIV antibody Any non-hepatic acute or chronic condition that, in the opinion of the Investigator, would limit the patient's ability to complete and/or participate in the study or compromise the integrity of the data Has experienced an illness that is considered by the Investigator to be clinically significant within 2 weeks before administration of investigational product Clinically relevant drug or alcohol abuse within 6 months of Screening. A positive drug screen will exclude subjects unless it can be explained by a prescribed medication Use of obeticholic acid (OCA), any drug of the same class, or fibrates (e.g., bezafibrate, fenofibrate, elafibranor, lanifibranor, pemafibrate, saroglitizar) within 30 days of Baseline Use of an experimental or unapproved treatment for PBC within 30 days of Baseline Clinically evident complication(s) of cirrhosis and portal hypertension that required either emergency room visit, hospital admission or both during the 12 week period prior to investigational product administration