A Phase 3, Open-Label, Long-Term Safety Extension Study Evaluating the Safety and Tolerability of the Fixed-Dose Combination of Obeticholic Acid and Bezafibrate in Subjects with Primary Biliary Cholangitis

Prior treatment

• Key Exclusion include: if they have a history or presence in the last 30 days, before rolling over to this study, of other concomitant liver diseases, varices, ascites/hepatic hydrothorax, spontaneous bacterial peritonitis, hepatic encephalopathy, or jaundice. Clinical complication of PBC including prior liver transplantation. Biochemical evidence of hepatic impairment, decompensation, or injury. Medical conditions that may cause non-hepatic increases in ALP. Presence of any other disease or condition that interferes with the absorption, distribution, metabolism, or excretion of drugs including bile salt metabolism in the intestine. History of cholelithiasis or choledocholithiasis unless documented cholecystectomy, chronic pancreatitis/recurrent acute pancreatitis, drug-induced myopathy, chronic kidney disease or undergoing dialysis, HIV, clinically concerning cardiac arrhythmias, severe pruritus, or hypersensitivity to OCA, BZF. Please refer to the Protocol for detailed Principal exclusion criteria.

• Key Target Population: All subjects with PBC who participated and are actively taking investigational product in Study 747-213 or Study 747-214 (or other Sponsor studies) are included. Please refer to the Protocol for detailed Principal inclusion criteria.