Primary sclerosing cholangitis

Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunc...

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Bibliographic Details
Published in:The Lancet (British edition) Vol. 391; no. 10139; pp. 2547 - 2559
Main Authors: Dyson, Jessica K, Beuers, Ulrich, Jones, David E J, Lohse, Ansgar W, Hudson, Mark
Format: Journal Article
Language:English
Published: England Elsevier Ltd 23.06.2018
Elsevier Limited
Subjects:
Bile
Bile ducts
Biomarkers
Cholangiocarcinoma
Cholangitis
Cholangitis, Sclerosing - diagnosis
Cholangitis, Sclerosing - etiology
Cholangitis, Sclerosing - pathology
Cholangitis, Sclerosing - therapy
Collaboration
Complications
Diagnostic systems
Disease Progression
Environmental factors
Fatigue
Fibrosis
Gallbladder diseases
Hepatitis
Humans
Hypertension
Inflammatory bowel disease
Inflammatory bowel diseases
Intestine
Liver
Liver diseases
Liver transplantation
Malignancy
Medical prognosis
Medical screening
Microbiomes
Pain
Patients
Phosphatase
Population
Pruritus
Surveillance
Transplantation
Transplants & implants
Vitamin deficiency
United Kingdom > UK
ISSN:0140-6736, 1474-547X, 1474-547X
Online Access:Get full text
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Description
Summary:Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography. Genetic and environmental factors are important in the cause of the disease, with the intestinal microbiome increasingly thought to play a pathogenetic role. Approximately 70% of patients have concurrent inflammatory bowel disease and patients require colonoscopic screening and surveillance. Primary sclerosing cholangitis is associated with increased malignancy risk and surveillance strategies for early cholangiocarcinoma detection are limited. No single drug has been proven to improve transplant-free survival. Liver transplantation is effective for advanced disease but at least 25% of patients develop recurrent disease in the graft.
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ISSN:0140-6736
1474-547X
1474-547X
DOI:10.1016/S0140-6736(18)30300-3