Primary biliary cholangitis

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic, progressive cholestatic liver disease characterised by destruction of small intrahepatic bile ducts, eventually leading to fibrosis and potential cirrhosis. Patients are usually middle-aged women and presents in the fifth or sixth decade of life. The most common symptoms are pruritus. It is not clear what causes PBC. It may be relate to problems in the autoimmune mechanism. The increased risk of genetic predisposition and environmental factors (infectious or toxic agents) trigger the disease. Ursodeoxycholic acid is currently the only medicine approved for the treatment of the disease.