Wechsler B, Le Thi Huong D U, Piette J C, Fassin D, Bletry O, Godeau P
Service de Médecine interne, C.H.U. Pitié-Salpêtrière, Paris.
J Mal Vasc. 1988;13(3):240-4.
Venous lesions in Behçet's disease (BD) were defined by Adamantiades and represent one of the most suggestive signs of the disease. They are occasionally the first sign of the disease and are frequently the basis for the diagnosis in a case of recurrent thrombosis in a young subject, the preferential context of BD. Involvement of superficial vessels is virtually constant. Venous vasculitis is responsible for non-specific hypersensitivity and erythema nodosa, which constitute some of the major diagnostic criteria. Ocular periphlebitis is one of the elements responsible for posterior uveitis. The originality of the venous involvement is due to the involvement of deep territories. Any vein may be affected, but the remarkable features are the size of the thrombosed vessels: superior and inferior vena cava, iliofemoral veins and the unusual site of the involvement: supra-hepatic veins, cerebral vessels, etc. Inferior vena cava thrombosis may be associated with aneurysms of the pulmonary arteries in the context of Hughes-Stovin syndrome. Cerebral phlebitis, which can now be identified more easily by means of digital angiography, is responsible for a typical picture: headaches, bilateral papilloedema and raised CSF pressure. The classical pictures of optic chiasmatic arachnoiditis and so-called benign intracranial hypertension actually correspond to unrecognised phlebitis. They may also be associated with other neurological lesions. In one half of cases, phlebitis cutaneous manifestations. However, they may precede the diagnostic signs or may occur very late in the course of the disease. They are recurrent and affect a number of different territories.(ABSTRACT TRUNCATED AT 250 WORDS)
白塞病(BD)中的静脉病变由阿达曼蒂亚德斯定义,是该疾病最具提示性的体征之一。它们偶尔是疾病的首发体征,在年轻患者反复发生血栓形成(BD的常见情况)时,常是诊断的依据。浅表血管受累几乎是恒定的。静脉血管炎导致非特异性超敏反应和结节性红斑,这是一些主要诊断标准的组成部分。眼部静脉周围炎是后葡萄膜炎的病因之一。静脉受累的独特之处在于深部区域也会受累。任何静脉都可能受到影响,但显著特征是血栓形成血管的大小:上、下腔静脉、髂股静脉,以及受累的不寻常部位:肝上静脉、脑血管等。在下腔静脉血栓形成的情况下,可能会在休斯-斯托文综合征的背景下出现肺动脉瘤。现在通过数字血管造影更容易识别的脑静脉炎会导致典型症状:头痛、双侧视乳头水肿和脑脊液压力升高。经典的视交叉蛛网膜炎和所谓的良性颅内高压实际上对应未被识别的静脉炎。它们也可能与其他神经病变相关。在一半的病例中,静脉炎有皮肤表现。然而,它们可能先于诊断体征出现,或者在疾病过程中很晚才出现。它们会反复出现,并累及多个不同区域。(摘要截选至250字)