Primary Sclerosing Cholangitis

This review summarizes the current understanding of primary sclerosing cholangitis, a persistent, progressive disease for which there is no definitive therapy. The authors describe the pathogenesis and management of this condition. Primary sclerosing cholangitis is an idiopathic, heterogeneous, chol...

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Bibliographic Details
Published in:The New England journal of medicine Vol. 375; no. 12; pp. 1161 - 1170
Main Authors: Lazaridis, Konstantinos N, LaRusso, Nicholas F
Format: Journal Article
Language:English
Published: United States Massachusetts Medical Society 22.09.2016
Subjects:
Bile
Cholangitis
Cholangitis, Sclerosing - classification
Cholangitis, Sclerosing - diagnosis
Cholangitis, Sclerosing - etiology
Cholangitis, Sclerosing - therapy
Disease management
End Stage Liver Disease - etiology
Liver diseases
Liver Transplantation
Pathogenesis
Ursodeoxycholic Acid - therapeutic use
United States > US
Netherlands
ISSN:0028-4793, 1533-4406, 1533-4406
Online Access:Get full text
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Summary:This review summarizes the current understanding of primary sclerosing cholangitis, a persistent, progressive disease for which there is no definitive therapy. The authors describe the pathogenesis and management of this condition. Primary sclerosing cholangitis is an idiopathic, heterogeneous, cholestatic liver disease that is characterized by persistent, progressive, biliary inflammation and fibrosis. There is no effective medical therapy for this condition. 1 End-stage liver disease necessitating liver transplantation may ultimately develop in affected patients. 2 The cause and pathogenesis of primary sclerosing cholangitis are unclear, although it is generally accepted that both genetic and environmental risk factors contribute to the development of the disease as well as to its progression and outcomes. 3 Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease (70 to 80% of patients have both conditions), and it is a . . .
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ISSN:0028-4793
1533-4406
1533-4406
DOI:10.1056/NEJMra1506330