Primary sclerosing cholangitis

Jessica K Dyson; Ulrich Beuers; David E J Jones; Ansgar W Lohse; Mark Hudson

doi:10.1016/S0140-6736(18)30300-3

Primary sclerosing cholangitis

Standard

Primary sclerosing cholangitis. / Dyson, Jessica K; Beuers, Ulrich; Jones, David E J; Lohse, Ansgar W; Hudson, Mark.

in: LANCET, Jahrgang 391, Nr. 10139, 23.06.2018, S. 2547-2559.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung

Harvard

Dyson, JK, Beuers, U, Jones, DEJ, Lohse, AW & Hudson, M 2018, 'Primary sclerosing cholangitis', LANCET, Jg. 391, Nr. 10139, S. 2547-2559. https://doi.org/10.1016/S0140-6736(18)30300-3

APA

Dyson, J. K., Beuers, U., Jones, D. E. J., Lohse, A. W., & Hudson, M. (2018). Primary sclerosing cholangitis. LANCET, 391(10139), 2547-2559. https://doi.org/10.1016/S0140-6736(18)30300-3

Vancouver

Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M. Primary sclerosing cholangitis. LANCET. 2018 Jun 23;391(10139):2547-2559. https://doi.org/10.1016/S0140-6736(18)30300-3

Bibtex

@article{7ab3230b78b34f2a870f8be8aa38ac64,

title = "Primary sclerosing cholangitis",

abstract = "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.",

keywords = "Journal Article, Review",

author = "Dyson, {Jessica K} and Ulrich Beuers and Jones, {David E J} and Lohse, {Ansgar W} and Mark Hudson",

year = "2018",

month = jun,

day = "23",

doi = "10.1016/S0140-6736(18)30300-3",

language = "English",

volume = "391",

pages = "2547--2559",

journal = "LANCET",

issn = "0140-6736",

publisher = "Elsevier Limited",

number = "10139",

}

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

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 -