Sciascia Savino, Radin Massimo, Bazzan Mario, Roccatello Dario
Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, S. Giovanni Bosco Hospital, Piazza del Donatore di Sangue 3, 10154, Turin, Italy.
SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy.
Intern Emerg Med. 2017 Feb;12(1):1-7. doi: 10.1007/s11739-016-1596-2. Epub 2017 Jan 2.
The anti-phospholipid syndrome (APS) is an autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity, associated with a persistent positivity for anti-phospholipid antibodies (aPL). The current classification criteria for APS include three laboratory tests: lupus anti-coagulant (LA), anti-cardiolipin (aCL), and anti-β2 glycoprotein-I (β2GPI). To date, the therapeutic approach for thrombotic APS mainly centers on long-term anti-coagulation with a vitamin K antagonist (VKA). APS management may represent a challenge for the treating physicians. Patients with different aPL profiles need a tailored risk-stratified approach. Moreover, in patients with recurrent thrombotic events despite therapy with VKA, or in those with microvascular involvement, new therapeutic options are highly needed. In this review, we aim to elucidate recent findings about new aPL specifities, available risk scoring models, and novel therapeutic approaches in APS management.
抗磷脂综合征（APS）是一种自身免疫性疾病，其特征为血管血栓形成和/或妊娠并发症，并伴有抗磷脂抗体（aPL）持续阳性。目前APS的分类标准包括三项实验室检查：狼疮抗凝物（LA）、抗心磷脂抗体（aCL）和抗β2糖蛋白I（β2GPI）。迄今为止，血栓性APS的治疗方法主要集中于使用维生素K拮抗剂（VKA）进行长期抗凝治疗。APS的管理对治疗医生而言可能是一项挑战。具有不同aPL谱的患者需要采用量身定制的风险分层方法。此外，对于尽管接受了VKA治疗仍发生复发性血栓事件的患者，或有微血管受累的患者，非常需要新的治疗选择。在本综述中，我们旨在阐明关于新的aPL特异性、可用的风险评分模型以及APS管理中的新型治疗方法的最新研究结果。
