Sclerosing cholangitis.

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 etiopathogene...

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Main Authors: MacFaul, G, Chapman, R
Format: Journal article
Language:English
Published: 2006
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author MacFaul, G
Chapman, R
author_facet MacFaul, G
Chapman, R
author_sort MacFaul, G
collection OXFORD
description 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.
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spelling oxford-uuid:f545cb6a-5f36-4703-9411-bc0629437e932022-03-27T12:26:05ZSclerosing cholangitis.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:f545cb6a-5f36-4703-9411-bc0629437e93EnglishSymplectic Elements at Oxford2006MacFaul, GChapman, R 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.
spellingShingle MacFaul, G
Chapman, R
Sclerosing cholangitis.
title Sclerosing cholangitis.
title_full Sclerosing cholangitis.
title_fullStr Sclerosing cholangitis.
title_full_unstemmed Sclerosing cholangitis.
title_short Sclerosing cholangitis.
title_sort sclerosing cholangitis
work_keys_str_mv AT macfaulg sclerosingcholangitis
AT chapmanr sclerosingcholangitis