Factors associated with thrombosis in Behçet Syndrome: A systematic review and meta-analysis.

OBJECTIVES

Thrombosis is an important component of vascular involvement in Behçet syndrome (BS). Inflammation seems to be the main cause, while the contribution of factors associated with thrombosis is debated.

METHODS

We searched PubMed and EMBASE for studies that assessed factors associated with thrombosis in patients with BS. We separately analyzed studies that compared BS patients with thrombosis to BS patients without thrombosis and studies that compared BS patients with thrombosis to non-BS patients with thrombosis. The pooled odds ratios with 95%CI were calculated for binary outcomes and standardized mean differences were calculated for continuous outcomes.

RESULTS

A total of 87 factors were compared between BS patients with thrombosis and BS patients without thrombosis in 101 studies. Having a Factor V Leiden mutation increased the risk of thrombosis 2.58 times (95% CI 1.76 to 3.78) among patients with BS. Homocysteine levels and factor VIII levels were also significantly higher among BS patients with thrombosis. There were only 6 studies including 14 factors that compared BS patients with thrombosis to non-BS patients with thrombosis. The frequencies of JAK-2 mutation, activated protein C resistance, levels of tissue plasminogen activator (tPA) and activity of tPA were significantly higher among non-BS patients with thrombosis.

CONCLUSION

Prothrombotic factors do not seem to be the main driver of thrombosis in BS, but may pose an additional risk when present. Screening BS patients with thrombosis for common prothrombotic factors may be reasonable, especially in patients with unusual clinical and demographic features for vascular involvement of BS.