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PURPOSE OF REVIEW: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree complicated by cirrhosis and cholangiocarcinoma. It is immune mediated although the precise etiology remains unknown. RECENT FINDINGS: Research into etiopathogenesis, epidemiology, the relationship with inflammatory bowel disease, diagnosis of cholangiocarcinoma, medical therapy, and the outcome of liver transplantation are discussed. SUMMARY: It is likely that a number of patients previously diagnosed with primary sclerosing cholangitis have autoimmune pancreatitis in association with primary sclerosing cholangitis, a syndrome with distinct clinicopathological features including steroid responsiveness. Primary sclerosing cholangitis-inflammatory bowel disease probably represents a distinct inflammatory bowel disease phenotype, which has implications for colonoscopic surveillance of these patients. CA19-9 plays no surveillance role for the early detection of cholangiocarcinoma. The best-studied drug in primary sclerosing cholangitis is ursodeoxycholic acid, which, despite a range of potentially valuable actions on the cholestatic liver, has not yet been proved to make a substantial impression on the course of the disease. Orthotopic liver transplantation remains the only established long-term treatment for primary sclerosing cholangitis.

Original publication

DOI

10.1097/01.mog.0000218966.78558.67

Type

Journal article

Journal

Curr Opin Gastroenterol

Publication Date

05/2006

Volume

22

Pages

288 - 293

Keywords

Bile Duct Neoplasms, Cholangiocarcinoma, Cholangitis, Sclerosing, Humans, Liver Transplantation