Autoimmune hepatitis: mecanisms, clinical, pathological and therapeutic features

Autoimmune hepatitis is characterized by an inflammation of the portal trac t with lymphocytes and plasma cells, an hypergammaglobulinemia and a variet y of circulating autoantibodies. The presence of smooth muscle antibodies and/or antinuclear antibodies defi ne type 1. Type 2 is characterized by the presence of liver-kidney - micros omal antibodies. Environmental, genetic and infectious factors may explain the autoreactivity of T cells. Different non specific clinical features may be present. Sometimes the presentation may be an acute hepatitis; in the r emainder, the disease may not be recognized until liver damage is advanced. Hypergammaglobulinemia and presence of circulating autoantibodies are the key for diagnosis. The association of prednisolone in combination with azat hioprine remains the established treatment. If relapse or non response occu r, other immunosuppressive therapy such as cyclosporin may be useful. Liver transplantation is reserved for (sub)fulminant forms and end stage liver d isease.