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Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology but lymphocytic portal tract infiltration is suggestive of an immune-mediated basis for this disease. Associations with inflammatory bowel disease (IBD) especially ulcerative colitis (UC), and with particular autoimmune diseases, as well as the genetic associations further suggest PSC may be an immune-mediated disease. The immunogenetics of PSC have been the subject of active research and several 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 hence evoking an abnormal immune response.

Type

Journal article

Journal

World J Gastroenterol

Publication Date

07/06/2008

Volume

14

Pages

3350 - 3359

Keywords

Antigens, Bacterial, Autoantibodies, Autoimmune Diseases, Bile Ducts, Chemotaxis, Leukocyte, Cholangitis, Sclerosing, Epithelial Cells, Genetic Predisposition to Disease, Humans, Immunity, Cellular, Immunogenetics, Major Histocompatibility Complex, Membrane Transport Proteins, Pancreatitis, Risk Factors, T-Lymphocytes