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Purpose: Changes in concentrations of acute phase proteins in the serum of patients might be significant in the pathogenesis of Behçet disease. This report investigates the association between ocular disease activity and serum haptoglobin levels in patients with Behçet disease, and summarizes the current understanding of the correlation between acute phase proteins and Behçet disease based on both personal studies and data from the literature., Methods: Thirty patients with Behçet disease with ocular involvement and 15 healthy subjects were included in the study. Of the 30 patients, 14 had acute uveitis and 16 had inactive ocular involvement at the time of enrollment., Results: There was a significant difference in haptoglobin levels between the patients with active ocular disease and controls (p=0.0005). There was also a significant difference in haptoglobin levels between the patients with inactive ocular disease and control subjects (p<0.0001). However, no significant difference was observed among patients with active versus inactive uveitis with regard to serum haptoglobin levels., Conclusions: Higher serum haptoglobin levels in patients with Behçet disease compared to control subjects were obtained. However, elevated serum haptoglobin levels do not seem to be a risk factor for uveitis activity. Behçet disease is generally diagnosed by physical examinations and no laboratory marker has been widely accepted for follow-up of disease activity.