Assessment of Anticardiolipin antibodies, Circulating Lupus anticoagulant, Protein C, Protein S, Antithrombin III &Activated Protein C Resistance and Their Relation to Thomboembolic and Other Clinical Manifestations in Behcet's Disease

Abstract

Background: Venous and arterial thrombosis occurs in patients with Behcet’s disease and is associated with significant morbidity and mortality. Studies on a possible association between the occurrence of thrombosis and thrombophilia in patients with this disease have been controversial. The objective of this study was to assess the frequency and clinical relevance of anticardiolipin antibodies (aCL) & other thrombophilic factors and their relationship to thromboembolic & clinical manifestations in Behcet's disease (BD). Materials and methods: IgG, IgM and IgA anticardiolipin antibodies (aCL) isotypes, presence of circulating lupus anticoagulant(LAC), protein C, protein S, antithrombin III & activated protein C resistance were investigated in 25 patients with BD and 25 patients with various rheumatic diseases not known to be associated with venous or arterial thromboembolic phenomena served as controls. Twelve of the patients with BD (48%) had either deep vein thrombosis (8 patients), arterial thromboembolic phenomena (4 patients), or both (2 patients). Results: The IgA aCL elevated in14 (56%) patients with BD compared with one (4%) patient in the control group (P<0.01). IgG aCL levels were elevated in 13 (52%) patients with Behcet's disease (BD) compared with one (4%) patient in the control group(P<0.01).Also patients with BD do not have decreased protein S, or antithrombin III activity, activated protein C resistance, circulating lupus anticoagulant (LAC), or elevated LgM aCL. No significant differences were found between any variable in both groups. No association between elevated IgMaCL levels and venous or arterial thrombosis and no statistical correlation was found between any factor and clinical manifestations of the disease. Conclusion: A significant number of patients have elevated levels of IgA& IgG aCL but they are not associated with venous or arterial thrombosis. These results do not suggest a primary role for aCL in BD and do not support the role of coagulation abnormalities in the pathogenesis of thromboembolic complications of Behcet’s disease but suggest vascular inflammation as the main pathogenetic event in the vascular lesions in Behcet’s disease.