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Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology;however, lymphocytic portal tract infiltration is suggestive of an immune-mediated basis for PSC. Associations with inflammatory bowel disease--especially ulcerative colitis--and with other auto-immune diseases, together with genetic associations, further suggest that PSC may be an immune-mediated disease. The immunogenetics of PSC have been the subject of active research, and several human leukocyte antigen (HLA)- and non-HLA-associated genes have been implicated in the development of the disease. Lymphocytes derived from the inflamed gut may enter the liver via the enterohepatic circulation to cause hepatic disease.PSC may be triggered in genetically susceptible individuals by infections or toxins entering the portal circulation through a permeable colon and, therefore, evoking an abnormal immune response.

Original publication

DOI

10.1385/CRIAI:28:2:093

Type

Journal article

Journal

Clin Rev Allergy Immunol

Publication Date

04/2005

Volume

28

Pages

93 - 103

Keywords

Animals, Antigens, Bacterial, Autoantibodies, Bile Ducts, Cholangitis, Sclerosing, Epithelial Cells, HLA Antigens, Humans, Immunogenetics, Major Histocompatibility Complex, Receptors, Antigen, T-Cell, Receptors, Lymphocyte Homing