Anticoagulants and immunosuppressive therapy for thrombosis in Behçet's syndrome: a systematic review and meta-analysis

Abstract

Background: Behçet's syndrome is a systemic vasculitis commonly associated with thrombotic manifestations. The primary treatment of vascular Behçet's syndrome involves immunosuppression; however, the role of anticoagulation remains unestablished.

Objectives: We conducted a systematic review and meta-analysis to evaluate the effectiveness of immunosuppressives alone, anticoagulants alone, and combined immunosuppressives and anticoagulants in preventing thrombosis relapse in Behçet's syndrome.

Methods: A systematic search was conducted using PubMed, EMBASE, and Cochrane Library from inception until April 1, 2025. Original studies enrolling ≥10 Behçet's syndrome patients with venous or arterial thrombosis were included if they reported intervention-specific recurrence or relapse rates of thrombosis. The primary outcome was the pooled relapse rate for each intervention group (immunosuppressives alone, anticoagulants alone, and combined immunosuppressives and anticoagulants), calculated using a random-effects model with 95% CIs. Subgroup analyses were performed to compare the pooled rates among the groups.

Results: A total of 20 studies (19 retrospective and 1 prospective) were included. Ten studies (315 patients) provided disaggregated data for the meta-analysis. The pooled relapse rate in the group receiving immunosuppressives and anticoagulants combined (17.7%; 95% CI, 9.7%-30.1%; I² = 56%) was significantly lower than that of those who received immunosuppressives alone (43.7%; 95% CI, 25.8%-63.4%; I² = 63%; P = .017) or anticoagulants alone (54.9%; 95% CI, 0.322%-0.758%; I² = 48%; P = .004).

Conclusion: Combined immunosuppressives and anticoagulants were associated with lower risks of thrombosis relapse compared with either treatment alone in Behçet's syndrome-associated thrombosis.

Keywords: Anticoagulants; Behcet Syndrome; Immunosuppressive Agents; Thrombosis; Vasculitis.