hepatic and biliary disorders
Primary Biliary Cholangitis (PBC)
Key Points
Primary biliary cholangitis is a chronic, progressive cholestatic liver disorder that is caused by an autoimmune attack on small bile ducts and that occurs almost exclusively in women aged 35 to 70. PBC typically progresses to a terminal stage over 15 to 20 years. Suspect PBC if patients have unexplained elevated alkaline phosphatase and gamma-glutamyl transpeptidase but minimally abnormal aminotransferases, particularly if they have constitutional symptoms or manifestations of cholestasis (eg, pruritis, osteoporosis,vitamin Ddeficiency). Measure IgM and antimitochondrial antibody, and do imaging (to rule out extrahepatic biliary obstruction). Consider liver biopsy. Stop use of hepatotoxins (including alcohol), and treat withursodeoxycholic acid, which may delay the need for transplantation.Obeticholic acidis the second-line treatment. Fibrate can be added toursodeoxycholic acid.Obeticholic acidand fibrate should be avoided in decompensated cirrhosis. Transplantation is indicated for decompensated liver disease (uncontrolled variceal bleeding, refractory ascites, intractable pruritus, hepatic encephalopathy). 