Sclerosing cholangitis.

Journal article

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.

Authors: MacFaul, G; Chapman, R

• Publication status: Published
• Journal: Current opinion in gastroenterology
• Volume: 22
• Issue: 3
• Pages: 288-293
• Publication date: 2006-05-01
• DOI: 10.1097/01.mog.0000218966.78558.67
• EISSN: 1531-7056
• ISSN: 0267-1379
• Language: English
• Keywords: Cholangiocarcinoma; Bile Duct Neoplasms; Humans; Liver Transplantation; Cholangitis, Sclerosing