Objective: To evaluate the prevalence of thrombophilic factors known to induce intravascular clotting and to assess their relationship with thromboembolic complications and the other clinical manifestations in Behçet's disease (BD).

Methods: Twenty-five patients with established BD were studied. Twenty patients with rheumatic conditions not known to be associated with venous or arterial thromboembolic phenomena served as controls. Eight of the patients with BD (32%) had either deep vein thrombosis (6 patients), arterial thromboembolic phenomena (2), or both (2). All participants were tested for IgG and IgM anticardiolipin antibody (aCL) levels, the presence of circulating lupus anticoagulant (LAC), protein C, protein S, and antithrombin III activity, activated protein C resistance, and where appropriate factor V Leiden mutation.

Results: Elevated levels of IgG aCL were detected in 10 (40%) patients with BD compared to one (5%) in the control group (p = 0.012). No significant differences were noted in the other variables studied between the 2 groups. No statistically significant correlation was found between any variable and the clinical manifestations.

Conclusion: Patients with BD do not have decreased protein C, protein S, or antithrombin III activity, activated protein C resistance, circulating LAC, or elevated levels of IgM aCL. A significant number of patients have elevated levels of IgG aCL but they are not associated with venous or arterial thrombosis. No correlation was found between any variable and other clinical manifestations of the disease.

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