Autoimmune hepatitides--update 2004.

This update reports is primarily made to update papers published in 2002 and 2003. A selection had to be realized since a search of the literature yielded more than 1600 journal articles. The prevailing opinion of an international group is especially emphasized. Autoimmune hepatitides (AIH)--as the name already suggests it--are no disease entity. We prefer to classify them under the terms of typical and atypical autoimmune hepatitides. Typical autoimmune hepatitis can be subdivided into a type 1 (with antibodies against nuclear material and/or smooth muscle) and a type 2 (with antibodies against endoplasmic reticulum). In the case of atypical autoimmune hepatitides, the detected autoantibodies would have to be stated. The essential feature of an autoimmune hepatitis is the presence of various high-titer autoantibodies circulating in serum, especially such of the IgG class. Special mention is made of the very rare liver ribosomal antibodies (LRA), which are mainly directed against 80S liver ribosomes and point to a so-called virus-associated autoimmune hepatitis. Also discussed are a genetic disposition, which is still speculative in some points, and the existence of so-called autogenes (physiological, pathological/pathogenic and so-called regulator genes). Various noxae, e.g. viruses, might also be "autogenous". Further research in the field of the autoimmune hepatitides (and also autoimmunity and autoimmune diseases as such) is therefore likely to concentrate on viruses and genes and their interrelations.