Autoimmune hepatitis (AIH) represents a chronic, mainly periportal hepatitis upon histology, which is characterized by a female predominance, hypergamma-globulinemia, circulating autoantibodies, and a benefit from immunosuppres-sive treatment. The diagnosis of AIH is based on clinical, serological, and im-munological features as well as on the exclusion of other hepatobiliary diseases with and without autoimmune phenomena. These include disease entities such as chronic hepatitis C, primary biliary cirrhosis, primary sclerosing cholangitis, and the so-called overlap or outlier syndromes. The revised AIH diagnostic score contributes to the establishment of the diagnosis in difficult cases by calculating a probability expressed as a numeric score .
Since the first description of AIH in 1950 by Waldenstrom , serological findings have attracted considerable attention not only for the diagnosis of this chronic liver disease but also as a means to study and eventually to understand its pathophysiology. Furthermore, serological detection of autoantibodies is a distinguishing feature that has been exploited for the subclassification of autoimmune hepatitis into three groups: AIH type 1, AIH type 2 and AIH type 3.
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