Position paper of the Italian Association for the Study of the Liver (AISF): Management and treatment of primary biliary cholangitis
Marco Carbone, Alessio Gerussi, Vincenzo Cardinale, N. Cazzagon, Valentina Cossiga, Ana Lleò, Giuseppe Marrone, Marco Marzioni, Antonio Moschetta, L. Muratori, Cristina Rigamonti, Umberto Vespasiani‐Gentilucci, Mirella Fraquelli, Vincenza Calvaruso
Abstract
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.