RT Book, Section A1 Dienstag, Jules L. A2 Jameson, J. Larry A2 Fauci, Anthony S. A2 Kasper, Dennis L. A2 Hauser, Stephen L. A2 Longo, Dan L. A2 Loscalzo, Joseph SR Print(0) ID 1178426963 T1 Autoimmune Hepatitis T2 Harrison's Principles of Internal Medicine, 20e YR 2018 FD 2018 PB McGraw-Hill Education PP New York, NY SN 9781259644016 LK accesspharmacy.mhmedical.com/content.aspx?aid=1178426963 RD 2024/04/18 AB Autoimmune hepatitis is a chronic disorder characterized by continuing hepatocellular necrosis and inflammation, usually with fibrosis, which can progress to cirrhosis and liver failure. When fulfilling criteria of severity, this type of chronic hepatitis, when untreated, may have a 6-month mortality of as high as 40%. Based on contemporary estimates of the natural history of autoimmune hepatitis, the 10-year survival is 80−98% for treated and 67% for untreated patients. The prominence of extrahepatic features of autoimmunity and seroimmunologic abnormalities in this disorder supports an autoimmune process in its pathogenesis; this concept is reflected in the prior labels lupoid and plasma cellhepatitis. Autoantibodies and other typical features of autoimmunity, however, do not occur in all cases; among the broader categories of “idiopathic” or cryptogenic chronic hepatitis, many, perhaps the majority, are probably autoimmune in origin. Cases in which hepatotropic viruses, metabolic/genetic derangements (including nonalcoholic fatty liver disease), and hepatotoxic drugs have been excluded represent a spectrum of heterogeneous liver disorders of unknown cause, a proportion of which are most likely autoimmune hepatitis.