Primary sclerosing cholangitis.
Primary sclerosing cholangitis (PSC) is a chronic bile duct disease leading to fibrotic biliary strictures and liver cirrhosis. The patient population is heterogeneous with regard to disease progression and the presence of co-morbidities, complicating the practical handling of patients as well as studies of pathogenetic mechanisms. The aetiology of PSC is unknown, but the recent findings of several robust susceptibility genes emphasise the importance of genetic risk factors. There is no effective medical treatment available to delay the disease progression, but endoscopic therapy of biliary stenoses may be indicated. Follow-up of patients includes management of the inflammatory bowel disease that is found in the majority of cases along with investigations aimed at the early detection of cholangiocarcinoma and colorectal cancer, which also occur at increased frequencies. In the present review, we aim to summarise the present knowledge of PSC with a particular emphasis on the possible basis of disease variability.
Karlsen
Tom H
TH
Norwegian PSC Research Center, Clinic for Specialized Medicine and Surgery, Oslo University Hospital, Rikshospitalet, 0027 Oslo, Norway. t.h.karlsen@klinmed.uio.no
Schrumpf
Erik
E
Boberg
Kirsten Muri
KM
D001650:Bile Duct Neoplasms / Q000453:epidemiology; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000453:epidemiology; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology; D015897:Comorbidity; D018450:Disease Progression; D005706:Gallbladder Neoplasms / Q000453:epidemiology; D020022:Genetic Predisposition to Disease; D055106:Genome-Wide Association Study; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000453:epidemiology; D015810:Linkage Disequilibrium; D008103:Liver Cirrhosis / Q000453:epidemiology / Q000503:physiopathology; D016031:Liver Transplantation; D017410:Practice Guidelines as Topic; D011379:Prognosis; D012307:Risk Factors; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D014580:Ursodeoxycholic Acid