Vascular involvement in Behcets disease
Behçet’s disease can involve all sizes of arteries and veins. Vascular disease is more common in men. Thrombosis of the large veins and arteries may occur, as can arterial aneurysms. Vascular thrombosis may be seen in a quarter of all patients and include thrombosis of the superior or inferior vena cava, portal or hepatic veins, and pulmonary arteries. Emboli from the thromboses are rare. Behçet’s disease patients with thrombosis may have the factor V Leiden mutation. Behçet’s disease is virtually alone among the vasculitides as a frequent cause of fatal aneurysms of the pulmonary arterial tree. Hughes–Stovin syndrome is a forme fruste of Behçet’s disease characterized by deep venous thrombosis and pulmonary artery aneurysms with hemoptysis being a common presenting manifestation. In addition to large-vessel involvement, superficial thrombophlebitis and small artery vasculitis can be seen.
