Ľubomír Gürtler
Autoimmune hepatitis are divided into the group of typical hepatitis and the group of variant forms or overlap syndromes. This illness is underdiagnosed. It occurs more frequently in women, and at any age but with two peaks (between 10-30 and 40-50 years of age). In etiopathogenesis conception of development („timeline“) of autoimmune disease is accepted and genetic predisposition is suggested. Triggers are environmental factors, mostly viruses, drugs and xenobiotics. Dysfunction at the level of regulatory T-cells (T reg) plays the key role. Clinical picture is variable, typical is fluctuation of disease activity, i.e. changing of periods of relapses and remissions. In 30 % of patients the disease is manifested as an acute hepatitis, in other group of patients it is presented as fulminate disease and in the rest of cases the onset of the disease is slowly, silent and the disease may be unrecognized. For diagnostics the score system is used that establishes definite or likely diagnosis. As no pathognomic features exist it is important to exclude other diseases. Strategy of immunosuppressive therapy is to get remission, avoid relapse and prevent progression of the disease into cirrhosis. Clinical and biochemical remission may be achieved very quickly after therapy. Retardation of histological remission after biochemical remission is 6-18 months. Two parameters predict the risk of relapse – the length of therapy and remission and histological activity of the disease. About 80-85 % of patients need long-term therapy because of repeated relapses. Appropriate immunosuppressive therapy is life preserving while wrong treated disease has usually bad prognosis. Heterogeneity of the disease requires individual approach.