Primary sclerosing cholangitis
Standard
Primary sclerosing cholangitis. / Dyson, Jessica K; Beuers, Ulrich; Jones, David E J; Lohse, Ansgar W; Hudson, Mark.
In: LANCET, Vol. 391, No. 10139, 23.06.2018, p. 2547-2559.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
Harvard
APA
Vancouver
Bibtex
}
RIS
TY - JOUR
T1 - Primary sclerosing cholangitis
AU - Dyson, Jessica K
AU - Beuers, Ulrich
AU - Jones, David E J
AU - Lohse, Ansgar W
AU - Hudson, Mark
N1 - Copyright © 2018 Elsevier Ltd. All rights reserved.
PY - 2018/6/23
Y1 - 2018/6/23
N2 - 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.
AB - 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.
KW - Journal Article
KW - Review
U2 - 10.1016/S0140-6736(18)30300-3
DO - 10.1016/S0140-6736(18)30300-3
M3 - SCORING: Review article
C2 - 29452711
VL - 391
SP - 2547
EP - 2559
JO - LANCET
JF - LANCET
SN - 0140-6736
IS - 10139
ER -