title: Epidemiology and treatment of autoimmune hepatitis.
abstract: Autoimmune hepatitis (AIH) is a chronic inflammatory disease of the liver that occurs worldwide with a low and probably underestimated prevalence. Although it typically affects young and middle-aged women, it can occur in both sexes and across all age groups. AIH runs a fluctuating course, but can present as severe and even fulminant hepatic failure or at a stage of advanced fibrosis or cirrhosis. Prognosis of severe AIH is poor if untreated. The pathogenesis is complex, combining environmental factors (external chemical or infectious triggers) and host genetic susceptibility. The diagnosis is based, after exclusion of other etiologies of chronic liver disease, on a combination of different elements, including the presence of elevated transaminases, elevated immunoglobulin G (IgG) levels, the presence and pattern of typical autoantibodies, and a liver biopsy showing interface hepatitis and other characteristic features. No single test can be used to make the diagnosis. Response to treatment can also help to establish the diagnosis. Simplified criteria can be used to make a bedside diagnosis with relatively high accuracy. Treatment consists of corticosteroids or other immunosuppressive regimens according to the severity of the disease, the response to the treatment, and the tolerance to therapy, with liver transplantation as an ultimate remedy in treatment-resistant cases with liver decompensation.
authors.affiliation: Antwerp University Hospital, Department of Gastroenterology Hepatology, Antwerp, Belgium ; Antwerp University, Faculty of Medicine and Health Sciences, Laboratory of Experimental Medicine and Paediatrics, Antwerp, Belgium.
authors.forename: Sven
authors.initials: S
authors.lastname: Francque
authors.affiliation: Antwerp University Hospital, Department of Gastroenterology Hepatology, Antwerp, Belgium ; Department of Internal Medicine, Immunology and Infectious Diseases, University of Bari, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Vonghia
authors.affiliation: Antwerp University Hospital, Department of Gastroenterology Hepatology, Antwerp, Belgium ; Institute and Laboratory for Genetic Diseases and Molecular Biology, Cologne, Germany.
authors.forename: Albert
authors.initials: A
authors.lastname: Ramon
authors.affiliation: Antwerp University Hospital, Department of Gastroenterology Hepatology, Antwerp, Belgium ; Antwerp University, Faculty of Medicine and Health Sciences, Laboratory of Experimental Medicine and Paediatrics, Antwerp, Belgium.
authors.forename: Peter
authors.initials: P
authors.lastname: Michielsen
keywords: antibodies; autoimmune hepatitis; epidemiology; pathophysiology; treatment