AIH type 1 represents the most common form of AIH (80% of all cases), mainly occurring between the ages of 16 and 30 years. The clinical course is usually not fulminant and an acute onset is very rare. It is characterized by the presence of antinuclear antibodies (ANAs) and/or anti-smooth muscle antibodies (SMAs). The target autoantigen(s) of type 1 autoimmune hepatitis are largely unknown but consist of multiple nuclear proteins including centromeres, ribonucleoproteins, and cyclin A as well as smooth muscle actin [13-17]. How-
13.3 Autoantibodies Frequently Associated with Autoimmune Hepatitis Type 1 293
ever, the molecular characterization of target antigen specificity does not supply important additional information to increase the diagnostic precision of AIH type 1. Although organ-specific autoantibodies are usually not observed, an association of AIH type 1 with other autoimmune syndromes is observed in 48% of cases, with autoimmune thyroid disease, synovitis, and ulcerative colitis as leading associations [18, 19].
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