The Pathophysiology of The Antiphospholipid Syndrome: A Perspective From The Blood Coagulation System.
The Pathophysiology of The Antiphospholipid Syndrome: A Perspective From The Blood Coagulation System.
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DOI：
10.1177/10760296221088576
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发表时间：
2022-01
期刊：
影响因子：
--
通讯作者：
Hernandez-Juarez J
中科院分区：
文献类型：
--
作者：
Arreola-Diaz R;Majluf-Cruz A;Sanchez-Torres LE;Hernandez-Juarez J
抗磷脂综合征（APS）是一种全身性自身免疫性疾病，其特征是与血管血栓形成和/或产科发病率相关的高凝性，由抗磷脂抗体如狼疮抗凝剂、抗β-2糖蛋白1和/或抗心磷脂抗体引起。在产科APS中，抗磷脂抗体诱导胎盘组织和募集的中性粒细胞产生促炎细胞因子和组织因子。此外，抗磷脂抗体激活补体系统，进而诱导正反馈，导致中性粒细胞的招募以及胎盘的激活。这些细胞的激活触发子宫肌收缩和宫颈成熟，从而诱发分娩。在血栓性和产科APS中，抗磷脂抗体激活内皮细胞、血小板和中性粒细胞，它们可能改变血管性血友病因子的多聚体模式和浓度，增加血栓反应蛋白1的浓度，减少抗凝血酶对因子XI的失活，增加因子XII的激活，并降低组织纤溶酶原激活剂的活性，随后产生纤溶酶。所有这些影响导致血栓渗透性降低，更致密，更薄，并且具有更多难以分解的支状纤维蛋白纤维。因此，血栓形成作为APS的临床标准，使患者的临床过程复杂化。
The antiphospholipid syndrome (APS), a systemic autoimmune disease characterized by a hypercoagulability associated to vascular thrombosis and/or obstetric morbidity, is caused by the presence of antiphospholipid antibodies such as lupus anticoagulant, anti-β-2-glycoprotein 1, and/or anticardiolipin antibodies. In the obstetrical APS, antiphospholipid antibodies induce the production of proinflammatory cytokines and tissue factor by placental tissues and recruited neutrophils. Moreover, antiphospholipid antibodies activate the complement system which, in turn, induces a positive feedback leading to recruitment of neutrophils as well as activation of the placenta. Activation of these cells triggers myometrial contractions and cervical ripening provoking the induction of labor. In thrombotic and obstetrical APS, antiphospholipid antibodies activate endothelial cells, platelets, and neutrophils and they may alter the multimeric pattern and concentration of von Willebrand factor, increase the concentration of thrombospondin 1, reduce the inactivation of factor XI by antithrombin, increase the activation of factor XII, and reduce the activity of tissue plasminogen activator with the subsequent production of plasmin. All these effects result in less permeable clots, denser, thinner, and with more branched fibrin fibers which are more difficult to lysate. As a consequence, thrombosis, the defining clinical criterion of APS, complicates the clinical course of the patient.
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DOI：
10.4049/jimmunol.1601219
发表时间：
2016-10-15
期刊：
Journal of immunology (Baltimore, Md. : 1950)
影响因子：
--
作者：
Appleby SL;Mitrofan CG;Crosby A;Hoenderdos K;Lodge K;Upton PD;Yates CM;Nash GB;Chilvers ER;Morrell NW
通讯作者：
Morrell NW
影响因子：
13.3
作者：
Abrahams, Vikki M.;Chamley, Lawrence W.;Salmon, Jane E.
通讯作者：
Salmon, Jane E.
影响因子：
5
作者：
Chen, Pojen P;Giles, Ian
通讯作者：
Giles, Ian
影响因子：
4.1
作者：
Chiu, Wen-Chin;Chiou, Tzeon-Jye;Chiang, An-Na
通讯作者：
Chiang, An-Na
影响因子：
20.3
作者：
Cugno, M;Cabibbe, M;Mannucci, PM
通讯作者：
Mannucci, PM