Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Viale Benedetto XV, 6, 16132, Genoa, Italy.
Centre of Excellence for Biomedical Research, University of Genoa, Genoa, Italy.
Clin Exp Med. 2017 Aug;17(3):257-267. doi: 10.1007/s10238-016-0430-5. Epub 2016 Jun 22.
Antiphospholipid antibody syndrome (APS) is an autoimmune acquired thrombophilia characterized by recurrent thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). APS can be primary, if it occurs in the absence of any underlying disease, or secondary, if it is associated with another autoimmune disorder, most commonly systemic lupus erythematosus. The exact pathogenetic mechanism of APS is unknown, but different, not mutually exclusive, models have been proposed to explain how anti-PL autoantibodies might lead to thrombosis and pregnancy morbidity. Diagnosis of APS requires that a patient has both a clinical manifestation (arterial or venous thrombosis and/or pregnancy morbidity) and persistently positive aPL, but the clinical spectrum of the disease encompasses additional manifestations which may affect every organ and cannot be explained exclusively by a prothrombotic state. Treatment for aPL-positive patients is based on the patient's clinical status, presence of an underlying autoimmune disease, and history of thrombotic events. In case of aPL positivity without previous thrombotic events, the treatment is mainly focused on reduction of additional vascular risk factors, while treatment of patients with definite APS is based on long-term anticoagulation. Pregnancy complications are usually managed with low-dose aspirin in association with low molecular weight heparin. Refractory forms of APS could benefit from adding hydroxychloroquine and/or intravenous immunoglobulin to anticoagulation therapy. Promising novel treatments include anti-B cell monoclonal antibodies, new-generation anticoagulants, and complement cascade inhibitors. The objective of this review paper is to summarize the recent literature on APS from pathogenesis to current therapeutic options.
抗磷脂抗体综合征（APS）是一种自身免疫性获得性血栓形成倾向，其特征是存在抗磷脂抗体（aPL）时反复发生血栓形成和妊娠并发症。如果 APS 发生在没有任何潜在疾病的情况下，则为原发性，如果它与另一种自身免疫性疾病相关，则为继发性，最常见的是系统性红斑狼疮。APS 的确切发病机制尚不清楚，但已经提出了不同的、非相互排斥的模型来解释抗 PL 自身抗体如何导致血栓形成和妊娠并发症。APS 的诊断需要患者既有临床表现（动脉或静脉血栓形成和/或妊娠并发症）又有持续阳性的 aPL，但该疾病的临床谱还包括其他可能影响每个器官的表现，不能仅通过促血栓形成状态来解释。针对 aPL 阳性患者的治疗基于患者的临床状况、是否存在潜在自身免疫性疾病以及血栓形成事件的病史。对于无先前血栓形成事件的 aPL 阳性患者，治疗主要集中于减少额外的血管危险因素，而对于明确的 APS 患者，治疗基于长期抗凝。妊娠并发症通常通过小剂量阿司匹林联合低分子量肝素进行管理。对于难治性 APS，可以在抗凝治疗的基础上添加羟氯喹和/或静脉注射免疫球蛋白。有前途的新型治疗方法包括抗 B 细胞单克隆抗体、新一代抗凝剂和补体级联抑制剂。本文的目的是总结最近关于 APS 的文献，从发病机制到当前的治疗选择。
