Position paper of the Italian Association for the Study of the Liver (AISF): Management and treatment of primary biliary cholangitis

Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic liver disease characterized by immune-mediated inflammation and destruction of the intrahepatic, small bile ducts. In PBC patients untreated or under-treated a progressive cholestasis leads to biliary fibrosis and ultimately liver cirrhosis. Disease presentation may differ within a wide spectrum, particularly based on the stage of the disease at onset; from asymptomatic, in most cases, or symptomatic, typically with pruritus and/or fatigue; jaundice may be present at presentation due to late diagnosis at cirrhotic stage or due to a premature severe ductopenia.