Behçet Disease With Vascular Involvement

Vascular involvement is one of the major causes of mortality and morbidity in Behçet disease (BD). There are no controlled studies for the management of vascular BD (VBD), and according to the EULAR recommendations, only immunosuppressive (IS) agents are recommended. In this study, we aimed to investigate the therapeutic approaches chosen by Turkish physicians during the initial event and relapses of VBD and the association of different treatment options with the relapses retrospectively. Patients with BD (n1⁄4 936, female/male: 347/589, mean age: 37.6 10.8) classified according to ISG criteria from 15 rheumatology centers in Turkey were included. The demographic data, clinical characteristics of the first vascular event and relapses, treatment proünyamin Kısacık, sut Onat, MD, Haner Direskeneli, MD presenting sign of the disease. After the first vascular event, ISs were given to 88.8% and AC treatment to 59.8% of the patients. Median duration of AC treatment was 13 months (1–204) and ISs, 22 months (1–204). Minor hemorrhage related to AC treatment was observed in 7 (4.7%) patients. A second vascular event developed in 32.9% (n1⁄4 86) of the patients. The vascular relapse rate was similar between patients taking only ISs and AC plus IS treatments after the first vascular event (29.1% vs 22.4%, P1⁄4 0.28) and was significantly higher in group taking only ACs than taking only ISs (91.6% vs 29.1%, P< 0.001). During follow-up, a third vascular event developed in 17 (n1⁄4 6.5%) patients. The relapse rate was also similar between the patients taking only ISs and AC plus IS treatments after second vascular event (25.3% vs 20.8%, P1⁄4 0.93). When multivariate analysis was performed, development of vascular relapse negatively correlated with only IS treatments. We did not find any additional positive effect of AC treatment used in combination with ISs in the course of vascular involvement in patients with BD. Severe complications related to AC treatment were also not detected. Our results suggest that short duration of IS treatments and compliance issues of treatment are the major problems in VBD associated with vascular relapses during follow-up. (Medicine 94(6):e494) Abbreviations: AC = anticoagulant, BD Behçet = disease, EULAR = European League Against Rheumatism, INR = international normalized ratio, IS = immunosuppressive, ISG = International Study Group, VBD = vascular Behçet disease. INTRODUCTION B ehçet disease (BD) is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions, and systemic involvement including gastrointestinal, musculoskeletal, neurological, and major vessels. Vasculitis is a main pathologic finding in BD. Vessels of all sizes can be involved, in both the arterial and venous systems, with venous and arterial occlusions and arterial aneurysms. Vascular involvement is observed in up to 40% of the patients with BD, especially in young men, and is one of the major causes of mortality and morbidity. Although venous thrombosis is seen primarily in the lower extremities, it may affect many different sites including the inferior and superior vena cava, pulmonary artery, suprahepatic vessels, and cardiac cavities. Up to 17% of the mortality in BD is reported to be involvement such as pulmonary embondrome. In a recent study from Turkey, % of patients with vascular BD (VBD) www.md-journal.com | 1 treatments were similar (P1⁄4 0.15 and P1⁄4 0.10). However, relapse was significantly lower in patients taking ISs (25.3% vs 85.7%, P< 0.001), whereas observed higher in the group TABLE 1. Clinical Characteristics of Vascular Behçet Disease (n1⁄4260) Gender Male (n1⁄4 224) 86.2% Age during first vascular event (years) 32.3 9.5 Only venous disease (n1⁄4 220) 84.6% Only arterial disease (n1⁄4 21) 8.1% Both venous and arterial disease (n1⁄4 11) 4.2% Cardiac involvement (n1⁄4 8) 3.1% Rare vascular involvements Budd–Chiari syndrome (n1⁄4 3) 1.2% Pulmonary aneurysm (n1⁄4 29) 11.2% Pulmonary thrombosis (n1⁄4 7) 2.7% Vena cava superior or inferior involvement (n1⁄4 22) 8.5% had recurrent vascular events in follow-up. New vascular event development risk was 23.0% at 2 years and 38.4% at 5 years. The primary pathology leading to venous thrombosis in BD is the inflammation of the vessel wall. Systemic immunosuppressives (ISs) are used to reduce this inflammation. However, there is no controlled study for the management of VBD. According to the EULAR recommendations for the management of BD, for acute deep vein thrombosis in BD, IS agents such as corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A are recommended. For the management of pulmonary and peripheral arterial aneurysms, cyclophosphamide and corticosteroids are recommended. Despite a high frequency of venous thrombosis, as pulmonary embolism is rare and a coexisting pulmonary aneurysm might result in fatal bleeding, anticoagulants (ACs), antiplatelet, or antifibrinolytic agents are not recommended. There are very limited data about anticoagulation in the treatment of deep venous thrombosis associated with BD. In this study, we aimed to investigate the therapeutic approaches chosen by Turkish physicians during the initial event and relapses of VBD and the association of different treatment options with the relapses, retrospectively.