Edwards C J, Hughes G R V
Department of Rheumatology, Southampton University Hospitals, Southampton, UK.
Mod Rheumatol. 2008;18(2):119-24. doi: 10.1007/s10165-008-0042-3. Epub 2008 Mar 4.
The antiphospholipid (Hughes) syndrome (APS) is a unique thrombotic disorder, causing both arterial and venous thrombosis, linked to the presence of antibodies directed against phospholipid-protein complexes. The first papers describing the syndrome were published in 1983 and, over the next two years, a series of publications described in detail the various clinical manifestations of the syndrome. Laboratory standardisation workshops were also set up and, in 1984, the first "world" symposium on APS was held. The international APS conferences have continued to grow in numbers and in stature. The APS has already had an impact in obstetrics, in medicine, in psychiatry, and in surgery. The approximate figure of 1 in 5 is a useful guide -- 1 in 5 of all young strokes, 1 in 5 recurrent miscarriages, 1 in 5 DVTs. More precise data will become available in the worlds of epilepsy, migraine, Alzheimer's, and MS. The advent of newer "biologic" immunosuppressives such as rituximab may offer help in selected cases. Intravenous immunoglobulin has proved successful, especially in the emergency setting.
抗磷脂（休斯）综合征（APS）是一种独特的血栓形成性疾病，可导致动脉和静脉血栓形成，与针对磷脂 - 蛋白质复合物的抗体的存在有关。描述该综合征的首批论文于1983年发表，在接下来的两年里，一系列出版物详细描述了该综合征的各种临床表现。还设立了实验室标准化研讨会，1984年举行了首届APS“世界”研讨会。国际APS会议的数量和规模持续增长。APS已经在产科、医学、精神病学和外科领域产生了影响。约五分之一这个大致数字是个有用的参考——所有年轻卒中患者中有五分之一、复发性流产患者中有五分之一、深静脉血栓形成患者中有五分之一。在癫痫、偏头痛、阿尔茨海默病和多发性硬化症领域将可获得更精确的数据。新型“生物”免疫抑制剂如利妥昔单抗的出现可能在某些病例中提供帮助。静脉注射免疫球蛋白已被证明是成功的，尤其是在紧急情况下。
