Inclusion Criteria

• Diagnosis of primary biliary cholangitis as demonstrated by the presence of ≥ 2 of the following 3 diagnostic criteria 
  • History of alkaline phosphatase elevated above upper limit of normal for at least 6 months 
  • Positive anti-mitochondrial antibodies (AMA) titer
    • Or if AMA negative or in low titer (<1:80) PBC specific antibodies (anti- GP210 and/or anti-SP100 and/or antibodies against the major M2 components (PDC-E2, 2-oxo-glutaric acid dehydrogenase complex) 
  • Previous liver biopsy consistent with PBC
• At least 1 of the following markers of disease severity
  • ALP ≥ 1.67× upper limit of normal (ULN)
  • Total bilirubin > ULN but < 2× ULN
• Must weigh at least 40 kg, and must have a body mass index (BMI) within the range of 18 - 35 kg/m^2
  • BMI = Body weight (kg) / [Height (m)]^2
• Able to communicate well with the investigator, to understand and comply with the requirements of the study

Exclusion Criteria

• Women of child-bearing potential, defined as physiologically capable of becoming pregnant, unless using highly effective methods of contraception during dosing and for 5 days following end of treatment
• History or presence of other concomitant liver diseases
• Clinically significant hepatic decompensation
• History of any venous thromboembolism, TIA, intracranial hemorrhage, neoplasm, arteriovenous malformation, vasculitis, bleeding disorder, coagulation disorders or screening blood tests that indicate altered coagulability (e.g. platelet count, aPTT, PTT or TT tests)
• History of conditions that may cause increases in ALP (e.g., Paget's disease)
• Use of other investigational drugs at the time of enrollment, or within 5 half-lives of enrollment