Summary

• More frequently affects women and has a variable course with different clinical manifestations, ranging from no symptoms to fulminant hepatic failure.
• Concurrent autoimmune diseases are often present.
• Aminotransferase levels are usually more strikingly raised than bilirubin and gamma-GT levels.
• Characterised by the presence of circulating auto-antibodies (ANA, smooth muscle antibody, perinuclear anti-neutrophil cytoplasmic auto-antibody, anti-soluble liver antigen/liver pancreas, anti-liver kidney microsomal-1, anti-liver cytosol) with a high serum globulin concentration and inflammatory changes on liver histology.
• Treatment options include corticosteroids alone or in combination with other immunosuppressants.
• Liver transplantation is indicated in patients with advanced liver disease who are refractory or intolerant to corticosteroid therapy.