Last update:
10-Sep-2004

Αutoimmune hepatitis is a chronic inflammatory liver disease associated with autoantibodies and high leves of serum globulin. The disease is more prevalent among females and may develop at any age but usually between the sixth and seventh decades of life. The clinical features cover a spectrum which extends from non-specific symptoms, the incidental findings of abnormal liver function tests during a screening examination or hepatosplenomegaly to acute or fulminant hepatitis or cirrhosis. Autoimmune hepatitis is a disease of unknown etiology but genetic factors seem to play a major role in its pathogenesis. Two types of autoimmune hepatitis have been proposed based on the circulating autoantibodies. Type I autoimmune hepatitis is characterized by the presence of antinuclear antibodies (ANA) and/or smooth muscle antibodies (SMA), antineutrophil cytoplasmic antibodies and, more ralely of antibodies against soluble liver antigens or liver-pancreas antigens (anti-SLA). Type II autoimmune hepatitis is characterized by the presence of antibodies to liver/kidney microsome type 1 (anti-LKM-1) and/or antibodies against liver cytosol 1 antigen (anti-LC1). In addition to the previously mentioned autoantibodies, other autoantibodies may also be detected; their presence further supports the diagnosis of autoimmune hepatitis or contributes in the reclassification of cryptogenic cases. Characteristics of both autoimmune hepatitis and primary biliary cirrhosis or autoimmune hepatitis and primary sclerosing cholangitis are observed in several overlapping syndromes. The treatment of autoimmune hepatitis is based on the administration of prednisone alone and/or other immunosuppressive drugs whenever there is an appropriate indication for therapy. The majority of patients respond satisfactorily to therapy, which prolongs survival, improves their quality of life and delays or even obviates the need for liver transplantation.

Key words: Antinuclear antibodies, Αntibodies to liver/kidney microsome, Autoimmune hepatitis, Cirrhosis, Smooth muscle antibodies .