Department of Medicine, Division of Rheumatology, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey.
Mediterr J Hematol Infect Dis. 2011;3(1):e2011026. doi: 10.4084/MJHID.2011.026. Epub 2011 Jul 8.
Behçet syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. The disease course is characterized by exacerbations and remissions while abating as the years pass. The usual onset is in the third decade. Recurrent skin mucosa lesions and sight threatening panuveitis are the hallmark of the disease. Males are more severely affected than females. Vascular involvement can occur in up to 40% of cases. BS is unique among the vasculitides in that it may involve all sizes and types of vessels. It affects the veins more than the arteries. Lower extremity vein thrombosis is the most frequent manifestation of vascular involvement, followed by vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms. Vascular involvement is frequently associated with constitut onal symptoms and increased acute phase response and is the major cause of increased mortality. A predominantly neutrophilic vasculitis around the vaso vasorum is typical of BS. The thrombus is tightly adherent to the vessel wall which probably explains why thromboembolism is so rare despite the high frequency of venous disease. Thrombophilic factors do not seem to explain thrombotic tendency in BS. Immunosuppressive treatment is essential in suppression and preventing the attacks.
贝赫切特综合征（BS）是一种多系统血管炎，病因不明，具有独特的地理分布。疾病过程的特点是随着时间的推移而缓解，反复发作。通常发病于第三个十年。反复发作的皮肤黏膜病变和威胁视力的全葡萄膜炎是该疾病的标志。男性比女性受影响更严重。高达 40%的病例会出现血管受累。BS 在血管炎中是独特的，因为它可能涉及所有大小和类型的血管。它对静脉的影响大于动脉。下肢静脉血栓形成是血管受累最常见的表现，其次是腔静脉血栓形成、肺动脉瘤、布加综合征、外周动脉瘤、硬脑膜窦血栓形成和腹主动脉瘤。血管受累常伴有全身症状和急性期反应增加，是导致死亡率增加的主要原因。BS 以血管周围以中性粒细胞为主的血管炎为特征。血栓紧密附着于血管壁，这可能解释了为什么尽管静脉疾病的发病率很高，但血栓栓塞却如此罕见。血栓形成倾向似乎不能用血栓形成倾向来解释。免疫抑制治疗对于抑制和预防发作至关重要。
