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...
Main Authors: | , |
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Format: | Journal article |
Language: | English |
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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. |
first_indexed | 2024-03-07T06:28:53Z |
format | Journal article |
id | oxford-uuid:f545cb6a-5f36-4703-9411-bc0629437e93 |
institution | University of Oxford |
language | English |
last_indexed | 2024-03-07T06:28:53Z |
publishDate | 2006 |
record_format | dspace |
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 |