Etiopathogenesis of primary sclerosing cholangitis

• Published in: World journal of gastroenterology : WJG Vol. 14; no. 21; pp. 3350 - 3359
• Main Authors: Chapman, Roger, Cullen, Sue
• Format: Journal Article
• Language: English
• Published: United States Department of Gastroenterology,John Radcliffe Hospital,Headington,Oxford OX3 9DU,United Kingdom 07.06.2008; The WJG Press and Baishideng
• Subjects: Antigens, Bacterial - immunology; Autoantibodies - blood; Autoimmune Diseases - genetics; Autoimmune Diseases - immunology; Autoimmune Diseases - microbiology; Bile Ducts - immunology; Chemotaxis, Leukocyte; Cholangitis, Sclerosing - genetics; Cholangitis, Sclerosing - immunology; Cholangitis, Sclerosing - microbiology; Epithelial Cells - immunology; Genetic Predisposition to Disease; Humans; Immunity, Cellular; Immunogenetics; Major Histocompatibility Complex - genetics; Major Histocompatibility Complex - immunology; Membrane Transport Proteins - genetics; Pancreatitis - immunology; Risk Factors; T-Lymphocytes - immunology; Topic Highlight; 免疫遗传学; 发病机理; 胆管炎; 自身抗体; Autoantibody; Immunogenetics; T cell receptor; Biliary epithelial cells; Lymphocytes
• ISSN: 1007-9327; 2219-2840
• DOI: 10.3748/wjg.14.3350

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.