Antiphospholipid Standardization Laboratory, Division of Rheumatology, Department of Internal Medicine, University of Texas Medical Branch, 301 University Boulevard, Galveston,TX 77555-0883, USA.
Semin Thromb Hemost. 2012 Jun;38(4):305-21. doi: 10.1055/s-0032-1311827. Epub 2012 Apr 17.
The presence of pathogenic antiphospholipid antibodies (aPL) is the characterizing feature of the antiphospholipid syndrome (APS), mediating the recurrent pregnancy loss and thrombosis typical of the disease, through their action on various antigenic targets. Despite the available knowledge regarding the mechanisms by which aPL induce a procoagulant phenotype in the vasculature and abnormal cellular proliferation and differentiation in placental tissues to cause the typical clinical features, these processes still remain incompletely understood. It is also known that inflammation serves as a necessary link between the observed procoagulant phenotype and actual thrombus development, and is an important mediator of the placental injury in APS patients. Even less well understood are the processes underlying the ontogeny of these pathogenic antibodies. This review seeks to highlight what is known about the mechanisms that contribute to the origin of pathogenic aPL and to the action of these antibodies on target antigens that produce the pathological features of APS. We will also examine the feasibility of classifying patients in clinical phenotypes related to underlying pathophysiological mechanisms, and how this could impact the management of patients with novel "targeted" therapeutic strategies.
致病性抗磷脂抗体 (aPL) 的存在是抗磷脂综合征 (APS) 的特征，通过其对各种抗原靶标的作用，介导疾病典型的复发性流产和血栓形成。尽管对于 aPL 如何在血管中诱导促凝表型以及在胎盘组织中引起异常细胞增殖和分化从而导致典型的临床特征有了相关的认识，但这些过程仍然不完全清楚。已知炎症是观察到的促凝表型和实际血栓形成之间的必要联系，也是 APS 患者胎盘损伤的重要介质。对于这些致病性抗体的发育过程，了解得更少。这篇综述旨在强调已知的有助于致病性 aPL 起源以及这些抗体对产生 APS 病理特征的靶抗原的作用的机制。我们还将检查根据潜在病理生理机制对患者进行临床表型分类的可行性，以及这将如何影响新型“靶向”治疗策略患者的管理。
