a Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine , Hokkaido University , Sapporo , Japan.
Expert Rev Clin Immunol. 2019 Jan;15(1):73-81. doi: 10.1080/1744666X.2019.1543025. Epub 2018 Nov 13.
Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia associated with the presence of persistent antiphospholipid antibodies (aPL). Owing to recent studies, not only APS patients but also incidentally-identified, asymptomatic aPL carriers are able to be stratified in terms of the risk of future thrombotic events, according to the variety and the titer of positive aPL tests and to the non-thrombotic, aPL-associated clinical manifestations. Areas covered: Here, we critically review (1) criteria manifestations of APS, (2) non-criteria manifestations of APS, (3) risk assessment in patients with APS and in aPL carriers, and (4) the potential role of primary thrombosis prophylaxis in aPL carriers. In addition, we discuss what we are currently able to do and what we need to do in the future for primary prophylaxis against a first thrombotic event. Expert commentary: We suggest a comprehensive algorithm to stratify thrombotic risk in aPL carriers, including criteria aPL, non-criteria aPL, their scoring systems, and non-criteria manifestations. However, further studies, particularly prospective randomized controlled trials, are highly warranted to establish an effective and tolerable treatment regimen for high risk aPL carriers.
抗磷脂综合征(APS)是一种获得性自身免疫性血栓形成倾向,与持续存在的抗磷脂抗体(aPL)有关。由于最近的研究,不仅 APS 患者,而且偶然发现的、无症状的 aPL 携带者,根据阳性 aPL 检测的种类和滴度以及与非血栓性、aPL 相关的临床表现,都能够对未来血栓事件的风险进行分层。
在这里,我们批判性地回顾了(1)APS 的标准表现,(2)APS 的非标准表现,(3)APS 患者和 aPL 携带者的风险评估,以及(4)原发性血栓预防在 aPL 携带者中的潜在作用。此外,我们还讨论了我们目前能够为预防首次血栓事件而进行的一级预防以及未来需要做的工作。
我们建议了一种综合算法,以对 aPL 携带者的血栓形成风险进行分层,包括标准 aPL、非标准 aPL、它们的评分系统和非标准表现。然而,仍需要进一步的研究,特别是前瞻性随机对照试验,以确定高风险 aPL 携带者的有效且可耐受的治疗方案。