title: Update on primary sclerosing cholangitis.
abstract: Primary sclerosing cholangitis (PSC) remains one of the most challenging conditions of clinical hepatology. There has been a steady growth in research to overcome this fact and the present review aims at summarizing the most recently published literature. The main emphasis will be put on the link of recent pathogenetic insights to clinical characteristics and patient management. With regard to pathogenesis, there is no consensus yet as to whether immune mediated injury or factors related to bile acid physiology are the most important. It also remains to be clarified whether PSC is a mixed bag of various secondary etiologies yet to be defined, or a disease entity predominantly represented by sclerosing cholangitis in the context of inflammatory bowel disease. Most important, there is no available medical therapy with proven influence on clinical end points, and timing of liver transplantation and patient follow-up are challenging due to the unpredictable and high risk of cholangiocarcinoma.
authors.affiliation: Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
authors.forename: Tom H
authors.initials: TH
authors.lastname: Karlsen
authors.forename: Kirsten Muri
authors.initials: KM
authors.lastname: Boberg
mesh_terms: D000818:Animals; D015551:Autoimmunity; D001647:Bile Acids and Salts / Q000378:metabolism / Q000627:therapeutic use / Q000633:toxicity; D001650:Bile Duct Neoplasms / Q000150:complications; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000150:complications; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D002779:Cholestasis / Q000209:etiology; D004064:Digestive System / Q000382:microbiology; D056726:Genetic Association Studies; D020022:Genetic Predisposition to Disease; D019693:Hepatitis, Autoimmune / Q000209:etiology; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism; D015212:Inflammatory Bowel Diseases / Q000150:complications; D016031:Liver Transplantation; D064307:Microbiota; D008954:Models, Biological; D011537:Pruritus / Q000209:etiology
chemical_list: D001647:Bile Acids and Salts; D007070:Immunoglobulin A
keywords: Cholangiocarcinoma; Inflammatory bowel disease; Primary sclerosing cholangitis