Autoimmune hepatitis: Pathogenesis, diagnosis and treatment

Background: Autoimmune hepatitis (AIH) is a chronic necro-inflammatory disease of the liver. Early recognition is important in order to prevent the development of cirrosis. This review discusses recent developments in the fields of diagnosis, pathophysiology and management of AIH. Methods: Relevant manuscripts were identified using an electronic database, and by hand search of a personal library. Results and conclusions: Description of new auto-antibodies, and formulation of diagnostic criteria and a scoring system by an international panel constitute important advances that may help diagnosis of the disease at an early stage. While a satisfying animal model of the disease is lacking, clinical observations have led to the formulation of a pathophysiological model. Current treatment has a failure rate of about 13%, and is unable to induce a permanent remission in most patients. New immunosuppressive agents (cyclosporine, tacrolimus and mycophenolate mofetil) appear promising, and should be evaluated in controlled trials.