Abstract
Vascular involvement is found in 6.3–51.6 % of the patients with Behçet’s disease (BD). It can be serious in some patients, especially in young-aged male patients. The lesions are distributed in the both arterial and venous systems, regardless of the size. Deep vein thrombosis (DVP) is the most characteristic, whereas superficial thrombophlebitis is considered as one of the cutaneous symptoms. DVP is located in any anatomical site, including superior and inferior vena cava, cerebral sinus, hepatic veins, and so on. Arterial lesions typically form aneurysms and occlusion. Peripheral arterial and pulmonary arterial aneurysms often lead to lethal events, whereas the occlusive lesions are negatively associated with remission. Cardiac involvement is also critical, though the incidence is rare. These lesions are illustrated by various imaging modalities such as angiography, CT scan, ultrasonography, MRI, and PET.
Because inflammation is the most important of underlying pathological changes in any type of vascular lesions, corticosteroids and immunosuppressants are used as first-line therapies. Recent reports have suggested that tumor necrosis factor (TNF) inhibitors are promising as an option. Surgical operation is necessary for impending rupture of aneurysm, though the procedures are frequently complicated with postoperative recurrence of aneurysm and occlusions. Endovascular intervention is an alternative with a lower incidence of complications. Use of anticoagulants and antiplatelets is controversial, because clinical efficacy of the agents has not been proven, and they may increase risk of fatal hemoptysis from pulmonary arterial aneurysms which frequently coexists.
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Takeno, M., Ideguchi, H., Suda, A., Kamiyama, R., Ishigatsubo, Y. (2015). Vascular Involvement of Behçet’s Disease. In: Ishigatsubo, Y. (eds) Behçet's Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54487-6_5
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