Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy

Core Tip

Core tip: Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies, and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. Ursodeoxycholic acid is the primary therapy. Obtecholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Complications of cirrhosis from advanced PBC include esophageal varices, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and hepatoma formation.