PMID- 22117638
OWN - NLM
STAT- MEDLINE
DCOM- 20120409
LR  - 20220317
IS  - 1532-1916 (Electronic)
IS  - 1521-6918 (Print)
IS  - 1521-6918 (Linking)
VI  - 25
IP  - 6
DP  - 2011 Dec
TI  - Pathogenesis of primary sclerosing cholangitis.
PG  - 727-39
LID - 10.1016/j.bpg.2011.10.009 [doi]
AB  - 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.
CI  - 2011 Elsevier Ltd. All rights reserved.
FAU - Pollheimer, Marion J
AU  - Pollheimer MJ
AD  - Division of Gastroenterology and Hepatology, Department of Internal Medicine, 
      Medical University of Graz, Austria.
FAU - Halilbasic, Emina
AU  - Halilbasic E
FAU - Fickert, Peter
AU  - Fickert P
FAU - Trauner, Michael
AU  - Trauner M
LA  - eng
GR  - P 19118/FWF_/Austrian Science Fund FWF/Austria
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - Netherlands
TA  - Best Pract Res Clin Gastroenterol
JT  - Best practice & research. Clinical gastroenterology
JID - 101120605
SB  - IM
MH  - Animals
MH  - Bile/chemistry
MH  - Cholangitis, Sclerosing/complications/*genetics/*immunology
MH  - Disease Models, Animal
MH  - Humans
MH  - Inflammatory Bowel Diseases/complications/immunology
MH  - Major Histocompatibility Complex/genetics
MH  - Mice
PMC - PMC3236286
EDAT- 2011/11/29 06:00
MHDA- 2012/04/10 06:00
PMCR- 2011/12/01
CRDT- 2011/11/29 06:00
PHST- 2011/10/11 00:00 [received]
PHST- 2011/10/25 00:00 [accepted]
PHST- 2011/11/29 06:00 [entrez]
PHST- 2011/11/29 06:00 [pubmed]
PHST- 2012/04/10 06:00 [medline]
PHST- 2011/12/01 00:00 [pmc-release]
AID - S1521-6918(11)00097-7 [pii]
AID - 10.1016/j.bpg.2011.10.009 [doi]
PST - ppublish
SO  - Best Pract Res Clin Gastroenterol. 2011 Dec;25(6):727-39. doi: 
      10.1016/j.bpg.2011.10.009.