Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, The Netherlands.
Hamostaseologie. 2010 Aug;30(3):139-43.
The antiphospholipid syndrome (APS) is a non-inflammatory autoimmune disease characterized by the presence of antiphospholipid antibodies (aPL) in the plasma of patients with vascular thrombosis, recurrent complications of pregnancy, or both (1, 2). The presence of aPL in plasma of patients can be detected with either a prolongation of phospholipid dependent coagulation tests (lupus anticoagulant, LAC), or with solid phase immune assays against the protein beta2-glycoprotein I (beta2-GPI) or the phospholipid cardiolipin (anti-beta2-GPI antibody ELISA and anti-cardiolipin antibody ELISA, respectively) (3). For a long time there was a lot of confusion on who had the syndrome and who not. To solve this dispute, an international consensus meeting was organized in Sapporo in 1999 to formulate classification criteria for patients with the antiphospholipid syndrome (4). These criteria have been updated in 2004 at another international consensus meeting in Sydney (5). The classification criteria were defined for scientific purposes and were aimed to be used as inclusion criteria in patient related studies. They were specifically not defined for diagnostic purposes. However, current practice is that these criteria are used as a diagnostic tool. This is very unfortunate because the specificity of the different aPL assays to detect the clinical manifestations that characterize APS are disputable. One of the aims of defining the criteria was to initiate studies to determine the value of the different anti-phospholipid antibody assays to serve as biomarker for the risk of thrombosis and pregnancy morbidity. The recent progress made on this important topic will be discussed.
抗磷脂综合征（APS）是一种非炎症性自身免疫性疾病，其特征是血管血栓形成、复发性妊娠并发症或两者同时存在的患者血浆中存在抗磷脂抗体（aPL）（1,2）。患者血浆中 aPL 的存在可以通过延长磷脂依赖性凝血试验（狼疮抗凝物，LAC）或固相免疫测定法针对蛋白质β2-糖蛋白 I（β2-GPI）或磷脂心磷脂（抗β2-GPI 抗体 ELISA 和抗心磷脂抗体 ELISA）来检测（3）。长期以来，谁患有该综合征，谁没有患有该综合征存在很多混淆。为了解决这一争议，1999 年在札幌组织了一次国际共识会议，制定了抗磷脂综合征患者的分类标准（4）。这些标准在 2004 年悉尼的另一次国际共识会议上进行了更新（5）。分类标准是为科学目的而制定的，旨在用作患者相关研究的纳入标准。它们不是专门为诊断目的而制定的。然而，目前的做法是将这些标准用作诊断工具。这是非常不幸的，因为不同的 aPL 检测方法检测到的特征性 APS 临床表现的特异性存在争议。定义这些标准的目的之一是启动研究，以确定不同抗磷脂抗体检测方法作为血栓形成和妊娠发病率风险的生物标志物的价值。将讨论在这一重要主题上取得的最新进展。
