Autoimmune hepatitis--an update

Autoimmune chronic active hepatitis (ai-CAH) is a distinct form of progressive inflammatory liver diseases of so far unknown etiology. If untreated it carries an unfavourable prognosis, therefore the diagnosis should be made as soon as possible. The diagnostic approach has been greatly facilitated by the establishment of a panel of marker autoantibodies that define several subgroups of the disease. Moreover the characterization of the targets of these autoantibodies shed new insight into the pathogenesis of the disease, even though the primary event triggering the disease remains to be defined. While the marker autoantibodies mentioned above are directed to intracellular structures additional immune reactions (both cellular and humoral) could be detected to organ specific components of the liver cell surface especially the asialoglycoprotein receptor (ASGPR). These latter reactions can be assumed to play a role in the initiation and/or perpetuation of the disease. Therapeutic strategies based on combined immunosuppression can considerably improve symptoms and prolong life expectancy. If they fail liver transplantation offers a very promising perspective.