Homocysteine in Vascular Behçet Disease

Abstract

Objective-Behçet disease (BD) is a vasculitis of unknown etiology, characterized by oral or genital aphtosis and uveitis.Homocysteine (hcy) is an independent risk factor for venous and arterial thrombosis. The association between hyperhomocysteinemia and thrombosis has been investigated in some studies in BD patients. However, information on this association is based only on the results of small studies with conflicting results. To overcome such limitations, we performed a metaanalysis comparing the plasma levels of hcy in BD patients with and without history of thrombosis. Methods and Results-We searched for all published studies using the Medline and Embase databases. Two reviewers performed study selection independently. Studies were included if hcy was measured in adult BD patients with and without thrombosis. Two reviewers independently extracted data on study and population characteristics. The mean value of hcy in BD patients and the presence of hyperhomocysteinemia in patients with and without thrombosis were collected. Association between thrombosis and hyperhomocysteinemia, and the mean difference of hcy levels in BD patients with and without thrombosis were calculated. Sixteen studies, for a total of 979 patients, were included. Hyperhomocysteinemia was more prevalent in patients with thrombosis than in those without (odds ratio 3.14; 95% CI 1.26 to 7.80) Mean levels of hcy were significantly higher in patients with thrombosis in comparison with patients without (mean difference 3.30 mol/L; 95% CI 2.09 to 4.51). This high prevalence of thromboses, known since the 1950s, 3 promoted several studies aimed at investigating their pathobiology. Endothelial injury itself, common to other vasculitis, cannot clearly account for thrombosis development, so a hypercoagulable state was early hypothesized and investigated. 4 In the last several years, several inherited or acquired factors causing hypercoagulability have been studied in patients with venous and arterial thromboembolic events. 5-7 These thrombophilic factors have also been studied in patients with BD with a history of thrombosis, and conflicting results were obtained. 8 -11 Recently, Ricart et al performed a metaanalysis evaluating the role of FV Leiden, prothrombin G2021A mutation, and the homozygous 677TT mutation of the methyltetrahydrofolate reductase as risk factors for venous thrombosis in BD patients. 12 In this study, FV Leiden and G2021A prothrombin mutation were found to increase the risk of thrombosis in these patients, whereas 677TT mutation of methyltetrahydrofolate reductase did not. Conclusion-OurThese findings are consistent with the results of recent studies performed in general populations in which the presence of 677TT mutation of methyltetrahydrofolate reductase is not a risk factor for venous thromboembolism in the absence of hyperhomocysteinemia. 13 On the other hand, in the general population, increased levels of homocysteine (hcy) have been reported to be an independent risk factor for venous thromboembolism in addition to c...

Serum Vitamin B12, Homocysteine and Methylmalonic Acid Levels in Patients With Parenchymal Neuro-Behçet’s Syndrome

“…16,17 Elevated Hcy has also been reported to be associated with thrombosis in vascular BS in several studies. [18][19][20][21] Cerebral venous sinus thrombosis frequently co-occurs with peripheral venous vascular events such as deep venous thrombosis and pulmonary vascular disease; therefore, serum Hcy levels are expected to be high in vascular NBS. 22 Homocysteine is known to be toxic for endothelial cells and has also been found to be associated with lower cognitive scores and neurological disorders.…”

Intracardiac Thrombus in a Young Man: Don't Forget Behçet's Disease!

“…Recently, La Regina and colleagues (22) showed via a large metaanalysis that hyperhomocysteinemia may be considered to be associated with thrombosis in BD patients and that the treatment with folic acid supplementation could improve the ophthalmic and vascular lesions.…”

“…Anticoagulants alone do not seem to be effective; systemic glucocorticoid and immunosuppressive agents are highly recommended before using anticoagulants (21). Surgery might be limited to the cases with massive thrombosis, recurrence of the complication despite an optimal medical treatment, and when there is a cardiac congestion (22).…”

Immunopathogenesis Based Adamantiades- Behcet Disease Vasculitis Treatment

“…Various pro-coagulant conditions, such as deficiencies of protein C, protein S, antithrombin III and factor V Leiden and prothrombin G20210A mutations have been described in ABD patients (Ates et al, 2003;Caramaschi et al, 2010). Additionally, higher levels of homocysteine, factor VIII, thrombin-antithrombin III complexes (TAT) and prothrombin fragment 1+2 maintain the intravascular generation of thrombin in these patients (Espinosa et al, 2002;La Regina et al, 2010). Despite all these data, it seems that thrombophilic factors do not play a major role in the thrombotic tendency of ABD.…”