Background: Autoimmune hepatitis (AIH) is a chronic inflammatory condition of the liver of unknown etiology. Its epidemiological and anatomoclinical characteristics and its outcome were unknown in Tunisia.

Aim: To analyse epidemiological, anatomoclinical, immunological and histological aspects of AIH and to determine factors predicting relapse after treatment and death of this disease in Tunisia.

Methods: Patients presenting with AIH between January 1996 and December 2004 were evaluated in retrospective multicentric study. The diagnosis of AIH was established according to the criteria of the revised score of the international autoimmune hepatitis group (1999)

Results: Eighty three patients were identified (70 female; mean age=49+17.9 years). 63% presented probable AIH and 37% presented definite AIH. Thirty two percent presented with the acute pattern. Eighty three per cent of cases were type I AIH and 5 % of cases were type II HAI. Fifty seven percent of the patients were cirrhotic at presentation. Associated autoimmune diseases was seen in 27 patients, dominated by diabetes, autoimmune thyroiditis and Sjögren's syndrome. An overlap syndrome was diagnosed in 25% of cases; primary biliary Cirrhosis-AIH in 20% of cases and primary sclerosing cholangitis-AIH in 5% of cases. Fifty patients were treated by glucocorticoids as monotherapy or in combination with azathioprine. Complete remission was achieved in 90% of cases. Fourteen percent relapsed within a median time of 12 months. Factors associated with relapse were: treatment with Azathioprine<18 months, absence of lobular necrosis and anti-nuclear antibody (+) profile. Mortality was observed in 17 % of cases. Factors associated with death were encephalopathy as an independent factor and treatment with Azathioprine<18 months.

Conclusion: In Tunisia, epidemiological and clinical characteristics of AIH were similar to those reported in the literature but with a higher frequency of cirrhosis at presentation. Treatment with Azathioprine < 18 months was the main factor associated with relapse and represented with encephalopathy a factor associated with death.

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