Clinical features and management of venous thromboembolism in patients with Behçet’s syndrome: a single-center case–control study

Almost one third of patients with Behçet's syndrome (BS) display vascular involvement. However, data regarding the prevalence and management of venous thromboembolism (VTE) in BS are scanty. We assessed the differential characteristics between patients with and without VTE and the factors associated with VTE incidence. A case–control study in a cohort of patients with BS was performed. 57 patients were included (56.1% women) with a mean follow-up of 10.56 (± 10.7) years. Mean age at diagnosis of BS and diagnosis of the first VTE episode was 34.7 (± 12.1) and 31.2 (± 8.9) years, respectively. Erythema nodosum (OR 4.6, CI 95% 1.2–18.1) and fever (OR 8.2, CI 95% 1.6–42.1) were associated with a higher risk of VTE. 26 episodes of VTE were registered in 12/57 (21%) patients. 83.3% of patients were not diagnosed with BS when the first episode of VTE occurred and, among them, the episode of VTE led to the diagnosis of BS in 40% of cases. Half of patients had at least one VTE recurrence. The absence of immunosuppressive treatment was associated with a higher risk of developing a first episode of VTE (OR 20 CI 95% 19.2–166.6). All patients were treated with anticoagulation and 75% were treated with immunosuppressants after the first VTE event. The diagnosis of VTE usually precedes that of BS, with a high frequency of VTE recurrence. Erythema nodosum and fever were associated with a higher risk of VTE, while the immunosuppressants showed a protective role for the development of VTE.