Wechsler B, Piette J C, Conard J, Huong Du L T, Blétry O, Godeau P
Presse Med. 1987 Apr 18;16(14):661-4.
A retrospective study of 177 patients (137 male, 40 female) with a disease that fulfilled the criteria of complete or incomplete Behçet's disease showed 106 thrombotic lesions of a venous trunk in 62 (35%) of these patients, superficial phlebitis and retinal vein thrombosis excluded. The incidence of thrombosis was unrelated to race and sex. The venous lesions were single in 38 cases and multiple in 24 cases, including 4 with more than 4 territories involved. Arterial lesions were also present in 7 cases. The lesions were located in the sural (n = 63), iliofemoral (n = 14), inferior vena cava (n = 13), superior vena cava (n = 3), axillary (n = 2) and cerebral (n = 13) veins or territories. Thrombosis developed during the first 4 years of Behçet's disease in one half of the patients. It preceded the aphthosis by 1 to 10 years in 11 cases, was concomitant with it in 15 cases and appeared more than 10 years after the diagnosis in 8 cases. Pulmonary embolism occurred in 11 patients, requiring clipping of the vena cava in 2, but it was never lethal. However, 2 of the 5 patients who died had venous thrombosis (Hughes Stovin syndrome). Thirty-eight patients could be followed up for 12 to 192 months (mean 43) under anticoagulant or antiplatelet therapy. Five vascular recurrences (13%) were observed, 2 of them (1 arterial graft thrombosis, 1 cerebral thrombosis) under anti-vitamin K agents. Cerebral thrombosis (9 men, 4 women) accounted for 27% of the neurological lesions; its prognosis was favourable in the 7 patients successfully treated with anticoagulants. Thrombosis could not be explained. Haemostasis studies showed a decrease of fibrinolytic activity compared to a group of normal subjects and an increase in fibrinogen, factor VIII and Willebrand's factor levels; these abnormalities did not seem to be specific. Thus, phlebitis truly is an element of Behçet's disease. Until its mechanism is better understood, effective anticoagulant therapy in cases with proven thrombosis and preventive antiplatelet therapy in the other cases seem to be rational measures.
对177例（137例男性，40例女性）符合完全或不完全白塞病标准的患者进行的回顾性研究显示，其中62例（35%）患者的静脉主干出现106处血栓形成病变，排除浅表性静脉炎和视网膜静脉血栓形成。血栓形成的发生率与种族和性别无关。静脉病变38例为单发，24例为多发，其中4例累及4个以上区域。7例患者还存在动脉病变。病变位于腓肠静脉（n = 63）、髂股静脉（n = 14）、下腔静脉（n = 13）、上腔静脉（n = 3）、腋静脉（n = 2）和脑静脉或脑区（n = 13）。一半的患者在白塞病发病的前4年内出现血栓形成。11例患者血栓形成先于口腔溃疡1至10年，15例与之同时出现，8例在诊断后10年以上出现。11例患者发生肺栓塞，2例需要进行腔静脉夹闭术，但均未致死。然而，死亡的5例患者中有2例患有静脉血栓形成（Hughes Stovin综合征）。38例患者在接受抗凝或抗血小板治疗后可随访12至192个月（平均43个月）。观察到5例血管复发（13%），其中2例（1例动脉移植血栓形成，1例脑血栓形成）发生在使用抗维生素K药物治疗期间。脑血栓形成（9例男性，4例女性）占神经病变的27%；7例成功接受抗凝治疗的患者预后良好。血栓形成的原因无法解释。止血研究显示，与一组正常受试者相比，纤溶活性降低，纤维蛋白原、因子VIII和血管性血友病因子水平升高；这些异常似乎并非特异性的。因此，静脉炎确实是白塞病的一个要素。在其机制得到更好理解之前，对于已证实有血栓形成的病例进行有效的抗凝治疗，以及对其他病例进行预防性抗血小板治疗似乎是合理的措施。
