Alijotas-Reig Jaume, Esteve-Valverde Enrique, Anunciación-Llunell Ariadna, Marques-Soares Joana, Pardos-Gea Josep, Miró-Mur Francesc
Systemic Autoimmune Diseases Research Unit, Vall d'Hebron Hospital Campus, Vall d'Hebron Institut de Recerca (VHIR), Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Vall d'Hebron Hospital Campus, Hospital Universitari Vall d'Hebron (HUVH), Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
J Clin Med. 2022 Jan 28;11(3):675. doi: 10.3390/jcm11030675.
Antiphospholipid syndrome is an autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity associated with persistent antiphospholipid antibody positivity. Cases fulfilling the Sydney criteria for obstetric morbidity with no previous thrombosis are known as obstetric antiphospholipid syndrome (OAPS). OAPS is the most identified cause of recurrent pregnancy loss and late-pregnancy morbidity related to placental injury. Cases with incomplete clinical or laboratory data are classified as obstetric morbidity APS (OMAPS) and non-criteria OAPS (NC-OAPS), respectively. Inflammatory and thrombotic mechanisms are involved in the pathophysiology of OAPS. Trophoblasts, endothelium, platelets and innate immune cells are key cellular players. Complement activation plays a crucial pathogenic role. Secondary placental thrombosis appears by clot formation in response to tissue factor activation. New risk assessment tools could improve the prediction of obstetric complication recurrences or thromboses. The standard-of-care treatment consists of low-dose aspirin and prophylactic low molecular weight heparin. In refractory cases, the addition of hydroxychloroquine, low-dose prednisone or IVIG improve pregnancy outcomes. Statins and eculizumab are currently being tested for treating selected OAPS women. Finally, we revisited recent insights and concerns about the pathophysiology, diagnosis and management of OAPS.
抗磷脂综合征是一种自身免疫性疾病,其特征为血管血栓形成和/或与持续抗磷脂抗体阳性相关的妊娠并发症。符合悉尼产科并发症标准但既往无血栓形成的病例被称为产科抗磷脂综合征(OAPS)。OAPS是反复妊娠丢失和与胎盘损伤相关的晚期妊娠并发症最常见的原因。临床或实验室数据不完整的病例分别归类为产科并发症抗磷脂综合征(OMAPS)和非标准OAPS(NC-OAPS)。炎症和血栓形成机制参与了OAPS的病理生理学过程。滋养层细胞、内皮细胞、血小板和固有免疫细胞是关键的细胞参与者。补体激活起着至关重要的致病作用。继发性胎盘血栓形成是由组织因子激活后形成凝块所致。新的风险评估工具可改善产科并发症复发或血栓形成的预测。标准治疗方案包括小剂量阿司匹林和预防性低分子肝素。在难治性病例中,加用羟氯喹、小剂量泼尼松或静脉注射免疫球蛋白可改善妊娠结局。目前正在对他汀类药物和依库珠单抗进行测试,用于治疗部分OAPS女性患者。最后,我们回顾了关于OAPS病理生理学、诊断和管理的最新见解和关注点。