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Background: There is very little information about autoimmune hepatitis (AIH) in populations other than of white European or Japanese descent., Goals: To investigate the presenting features, immunogenetic background, and response to standard immunosuppressive therapy in native Turkish patients with AIH., Methods: Retrospective analysis of all patients referred between 1994 and 2000 to our hepatology clinic in Ankara who fulfilled international criteria for definite or probable type 1 or type 2 AIH., Results: Seventeen patients (15 female; median age, 31 years; range, 13-56 years) were identified. All had type 1 AIH. Clinical and laboratory features were broadly similar to those reported for white or Japanese patients. Five had the HLA DR3 allotype and 10 had DR4; however, in contrast to white and Japanese patients, DR4 was not associated with an older age at onset. Importantly, no patient had the B8 allotype (vs. 10.9% in 110 healthy Turkish subjects). Thus, none had the classic A1-B8-DR3 autoimmune haplotype: a major distinction from white individuals., Conclusions: There appears to be genetic differences in type 1 AIH between Turkish and other populations. Genotyping of Turkish patients to identify alleles that may confer susceptibility or resistance to AIH may progress understanding of the genetic basis of the disease.