Although systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) are distinct diseases, in clinical practice differentiation of one from other may be difficult. The aim of this study was to asses features of SLE in patients with diagnosis of AIH.Thirty patients [mean age: 52.4 +/- A 11.8 years; 23 (76.7 %) female] were included in the study. Seven (23.3 %) of the patients full filled 4 or more criteria for classification of SLE. None of the patients had muco-cutaneous lesions characteristic to SLE. Three patients had rheumatoid factor negative arthritis, and 2 patients had pericardial effusion. Four patients had significant thrombocytopenia (< 100 x 10(3)/mu L), and one of these patients had pancytopenia. None of the patients had hematuria, but 3 patients had proteinuria which did not affect renal function during the study period. One patient died due to pancytopenia-associated pulmonary infection. Among the treated patients with SLE features, 2/5 (40 %) achieved ALT normalization and 9/12 (75 %) of the remaining patients achieved ALT normalization (Fisher's exact test; p = 0.28) during the study period. Although the difference is non-significant, treatment response of AIH patients with SLE features seemed to be delayed and incomplete compared to other patients, but with the limited number of patients it is inconvenient to reach a definitive conclusion. Further studies are needed to identify role of features of SLE on treatment response in patients with AIH.