Vascular manifestations of Behçet's disease. Eighteen cases among 140 patients

To describe the features, prognosis, and treatment of vascular involvement in Behçet's disease (BD). Among 140 patients with BD seen at the Hôtel-Dieu Hospital in Beirut between 1980 and 2000, 18 (13%) had vascular involvement and were included in this retrospective study. All these patients fulfilled International Study Group criteria for BD. Men with BD were more likely to have vascular involvement (13/77, 17%) than women (5/63, 8%) (P = 0.12) and were younger at diagnosis of vascular disease (32 +/- 7 vs. 36 +/- 7.5 years; P < 0.01). Many patients had vascular disease at more than one site: 17 had thrombophlebitis, 10 had arterial thromboses, and one had an aneurysm. Thrombophlebitis was more common in men (82% vs. 18%; P < 0.03) and arterial occlusion in women (70% vs. 30%; P > 0.05). Caval thrombosis and arterial occlusions were the most serious complications. Combined treatment with glucocorticoids, anticoagulants, and immunosuppressants was effective in superior vena cava syndrome and extracranial arterial occlusion. Vascular manifestations of BD are common in Lebanon, particularly venous lesions. Aneurysms are seen less often than arterial occlusions. Medical treatment may be sufficient in superior vena cava syndrome and arterial occlusion.