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Pollheimer, MJ; Halilbasic, E; Fickert, P; Trauner, M.
Pathogenesis of primary sclerosing cholangitis.
Best Pract Res Clin Gastroenterol. 2011; 25(6):727-739 Doi: 10.1016/j.bpg.2011.10.009 [OPEN ACCESS]
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Führende Autor*innen der Med Uni Graz
Pollheimer Marion
Trauner Michael
Co-Autor*innen der Med Uni Graz
Fickert Peter
Halilbasic Emina
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Abstract:
Primary sclerosing cholangitis (PSC) represents a chronic cholestatic liver disease with fibroobliterative sclerosis of intra- and/or extrahepatic bile ducts, eventually leading to biliary cirrhosis. The association with human leukocyte antigen (HLA) and non-HLA haplotypes and the presence of autoantibodies in sera of PSC patients support a crucial role for immune-mediated mechanisms in the initiation and progression of PSC. The strong clinical association between PSC and inflammatory bowel diseases led to intriguing pathogenetic concepts, in which the inflamed gut with translocation of bacterial products and homing of gut-primed memory T lymphocytes via aberrantly expressed adhesion molecules plays a fundamental role. Genetically or chemically modified bile composition was shown to induce sclerosing cholangitis and liver fibrosis in a number of animal models ("toxic bile concept"). The potential role of vascular injury with ischemia of bile duct epithelium cells in the development of sclerosing cholangitis is supported by animal models of endothelial cell injury showing close morphological similarities with human PSC.
Find related publications in this database (using NLM MeSH Indexing)
Animals -
Bile - chemistry
Cholangitis, Sclerosing - complications
Disease Models, Animal -
Humans -
Inflammatory Bowel Diseases - complications
Major Histocompatibility Complex - genetics
Mice -

Find related publications in this database (Keywords)
Primary sclerosing cholangitis (PSC)
Aetiology
Pathogenesis
Immunology
Inflammatory bowel diseases
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