摘要
Primary sclerosing cholangitis(PSC)is a chronic,progressive,hepatobiliary disease characterized by inflammation and fibrosis of the intra-and extra-hepatic bile ducts.Its natural history is one that generally progresses towards cirrhosis,liver failure,cholangiocarcinoma,and ultimately disease-related death,with a median liver transplantation-free survival time of approximately 15-20 years.However,despite its lethal nature,PSC remains a heterogenous disease with significant variability in outcomes amongst different regions of the world.There are also many regions where the outcomes of PSC have not been studied,limiting the overall understanding of this disease worldwide.In this review,we present the geoepidemiologic variations in outcomes of PSC,with a focus on survival pre-and post-liver transplantation as well as the concurrence of inflammatory bowel disease and hepatobiliary neoplasia.
