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Autoimmune liver disease

机译:Autoimmune liver disease

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The diagnostic criteria of autoimmune hepatitis have been codified, and concepts of autoantibody expression, genetic predisposition, immunopathogenesis, disease behavior, and treatment have been extended. A scoring system quantitates the net strength of the diagnosis, but it does not sufficiently downgrade laboratory and histologic features of cholestasis to be used generally. Antibodies to asialoglycoprotein receptor identify patients who are prone to relapse after corticosteroid withdrawal, and antibodies to actin characterize patients with a poorer immediate treatment response than seronegative counterparts. These antibodies may ultimately supplant conventional markers that lack prognostic implication. Antibodies to heat shock proteins have promise as indices of disease activity, and antineutrophil cytoplasmic antibodies have similar frequencies of low titer seropositivity in primary sclerosing cholangitis and autoimmune hepatitis. These latter antibodies also have similar immunoglobulin G subclasses, and they may have a common target antigen. The same genetic risk factors for autoimmune hepatitis are associated with immune manifestations in other chronic liver diseases, and the host predisposition rather than the nature of the liver disease may be the critical determinant of the immune response. Corticosteroidtreated patients with severe autoimmune hepatitis have the same 10-year survival as age- and gender-matched normal subjects and histologic cirrhosis at presentation does not diminish the immediate response to treatment or lower the 1 0-year life expectancy. 6-Mercaptopurine can enhance the efficacy of a prednisone regimen in patients in whom therapy with azathioprine has failed.

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