Sclerosing cholangitis.

PURPOSE OF REVIEW: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree. It is immune mediated, although the precise cause remains unknown. Recent reports have shown a higher prevalence and burden of disease than was previously suspec...

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Main Authors: MacFaul, G, Chapman, R
Format: Journal article
Language:English
Published: 2005
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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. It is immune mediated, although the precise cause remains unknown. Recent reports have shown a higher prevalence and burden of disease than was previously suspected. RECENT FINDINGS: The research into the etiopathogenesis, epidemiology, diagnosis of cholangiocarcinoma, medical and surgical therapy, and timing and outcome of liver transplantation is discussed. SUMMARY: Genetic heterogeneity among patients with primary sclerosing cholangitis is supported, and further gene polymorphisms associated with protection against primary sclerosing cholangitis have been elucidated. Bile duct injury seems to be a multistep process. Magnetic resonance cholangiopancreatography is a cost-effective and accurate way of diagnosing primary sclerosing cholangitis in comparison with endoscopic retrograde cholangiopancreatography. Ursodeoxycholic acid may have a role as a colorectal and hepatobiliary cancer chemopreventive agent. Liver transplantation remains the only treatment in end-stage disease. The 5-year and 10-year patient and graft survival rates are comparable with those in patients without primary sclerosing cholangitis, but there is a higher rate of retransplantation for primary sclerosing cholangitis in most centers. Hepatobiliary malignancy is found in a minority of patients at transplantation, although 5-year survival rates for these patients are still promising.
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spelling oxford-uuid:08fdc993-317a-49a5-8de8-d4f98d46aec72022-03-26T09:15:55ZSclerosing cholangitis.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:08fdc993-317a-49a5-8de8-d4f98d46aec7EnglishSymplectic Elements at Oxford2005MacFaul, GChapman, R PURPOSE OF REVIEW: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree. It is immune mediated, although the precise cause remains unknown. Recent reports have shown a higher prevalence and burden of disease than was previously suspected. RECENT FINDINGS: The research into the etiopathogenesis, epidemiology, diagnosis of cholangiocarcinoma, medical and surgical therapy, and timing and outcome of liver transplantation is discussed. SUMMARY: Genetic heterogeneity among patients with primary sclerosing cholangitis is supported, and further gene polymorphisms associated with protection against primary sclerosing cholangitis have been elucidated. Bile duct injury seems to be a multistep process. Magnetic resonance cholangiopancreatography is a cost-effective and accurate way of diagnosing primary sclerosing cholangitis in comparison with endoscopic retrograde cholangiopancreatography. Ursodeoxycholic acid may have a role as a colorectal and hepatobiliary cancer chemopreventive agent. Liver transplantation remains the only treatment in end-stage disease. The 5-year and 10-year patient and graft survival rates are comparable with those in patients without primary sclerosing cholangitis, but there is a higher rate of retransplantation for primary sclerosing cholangitis in most centers. Hepatobiliary malignancy is found in a minority of patients at transplantation, although 5-year survival rates for these patients are still promising.
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