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To evaluate the relationship between disease activity and levels of alpha 1-acid glycoprotein and pentraxin 3 in patients with Behçet's disease (BD).Forty-eight patients with BD and 29 age- and gender-matched healthy control subjects were included in the study. Serum pentraxin 3 levels were measured using enzyme-linked immunosorbent assay (ng/ml), and alpha 1-acid glycoprotein levels were measured using nephelometry (mg/dl). Disease activity was assessed using the BD Current Activity Form (BDCAF).Serum alpha-1 acid glycoprotein levels were significantly higher in patients with BD compared to the control group (P = 0.048). There were no significant differences between two groups in terms of levels of pentraxin 3 (P = 0.697). According to Pearson's analysis, alpha 1-acid levels are significantly positively correlated with erythrocyte sedimentation rate, C-reactive protein, and skin lesions and arthritis of BDCAF scores. Pentraxin 3 levels did not correlate with erythrocyte sedimentation rate, C-reactive protein, or any domains of BDCAF scores.The results of the present study demonstrate that serum levels of alpha-1 acid glycoprotein were significantly higher in patients with BD relative to the control group. Alpha-1 acid glycoprotein is greatly associated with skin lesions and arthritis in patients with BD. We did not find high serum levels of PTX3 in patients with BD compared to healthy controls, and pentraxin 3 is not associated with disease activity in BD.