Haemostasis Research Unit, Department of Haematology, University College London, London, UK.
Br J Haematol. 2014 Jun;165(5):609-17. doi: 10.1111/bjh.12848. Epub 2014 Mar 29.
Consideration of the chronology of advances in medical knowledge can provide useful insights into the pathogenesis, diagnosis and treatment of diseases. The antiphospholipid syndrome is an enigmatic disorder and this is reinforced by the misleading associated terminology, the adoption of which results directly from early discoveries relating to the condition. Thus the target antigen of the causative autoantibodies in antiphospholipid syndrome does not reside on phospholipid, and the frequently associated lupus anticoagulant is not restricted to subjects with systemic lupus erythematosus and, paradoxically, despite causing prolongation of clotting times in vitro it is associated with a pronounced tendency to thrombosis. Recognition of the antiphospholipid syndrome has its origins in the identification of subjects with so-called biological false-positive serological reactions for syphilis in the middle years of the last century. Since that time there have been considerable advances in our understanding of the pathogenesis of the disease and the clinical manifestations and associations, improved diagnostic accuracy and an evolving evidence base for optimal therapy. However many gaps in our knowledge remain.
考虑医学知识进展的时间顺序，可以为疾病的发病机制、诊断和治疗提供有用的见解。抗磷脂综合征是一种神秘的疾病，这一点因误导性的相关术语而更加明显，这些术语的采用直接源于与该疾病相关的早期发现。因此，抗磷脂综合征中致病自身抗体的靶抗原并不存在于磷脂上，而经常与之相关的狼疮抗凝物不仅局限于系统性红斑狼疮患者，而且具有矛盾的是，尽管它在体外会导致凝血时间延长，但却与明显的血栓形成倾向有关。抗磷脂综合征的认识起源于上个世纪中叶对所谓梅毒生物学假阳性血清学反应的患者的识别。自那时以来，我们对该疾病的发病机制、临床表现和关联、诊断准确性的提高以及最佳治疗方法的循证基础有了很大的了解。然而，我们的知识仍存在许多空白。
