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抗磷脂综合征发病机制、诊断与治疗的新认识。

Novel insights into pathogenesis, diagnosis and treatment of antiphospholipid syndrome.

机构信息

Lupus Research Unit, The Rayne Institute, King's College London School of Medicine, London, UK.

出版信息

Curr Opin Rheumatol. 2012 Sep;24(5):473-81. doi: 10.1097/BOR.0b013e328354ae8c.

DOI:10.1097/BOR.0b013e328354ae8c
PMID:22585233
Abstract

PURPOSE OF REVIEW

To provide an update of recent insights into the pathogenesis, diagnosis and treatment of antiphospholipid syndrome (APS) from current literature.

RECENT FINDINGS

β2GPI was recently implicated in the pathogenesis of thrombosis. High titres of anti-β2GPI antibodies are present in patients with triple positivity which highlight its importance. Consensus guidelines have been published to standardise diagnostic assays and once implemented may yield more accurate diagnoses of APS. An 'aPL score' has been formulated to improve the detection and outcomes of patients. New oral anticoagulants, statins and concomitant therapy with warfarin and aspirin have been identified as potential novel therapeutic interventions for thrombotic APS. Advances in the pathogenesis of obstetric APS have occurred, such as the concept of redefining the syndrome as inflammatory and clearer identification of the roles of complement, β2GPI and annexin 5. Independent risk factors for pregnancy failure have been recognised and when combined with clinical and laboratory features may improve patient outcomes. Interventions involving adjusted doses of low molecular weight heparin in combination with aspirin have shown promising results from initial studies.

SUMMARY

Recent insights into the pathogenesis of APS have unveiled novel areas for treatment intervention. Diagnostic criteria and recommendations have been revised and formulated to provide consensus and standardisation for diagnosis.

摘要

目的综述

从目前的文献中,提供抗磷脂综合征(APS)发病机制、诊断和治疗的最新研究进展。

最近的发现

β2糖蛋白 I(β2GPI)最近被认为与血栓形成的发病机制有关。三重阳性患者存在高滴度的抗β2GPI 抗体,这突出了其重要性。已经发表了共识指南来标准化诊断检测,一旦实施,可能会更准确地诊断 APS。制定了“aPL 评分”以提高对患者的检测和结果。新型口服抗凝剂、他汀类药物以及与华法林和阿司匹林的联合治疗已被确定为血栓性 APS 的潜在新型治疗干预措施。在产科 APS 的发病机制方面也取得了进展,例如将该综合征重新定义为炎症性疾病,以及更清楚地确定补体、β2GPI 和膜联蛋白 5 的作用。已经认识到与妊娠失败相关的独立危险因素,当与临床和实验室特征相结合时,可能会改善患者的结局。调整剂量的低分子量肝素联合阿司匹林的干预措施在初步研究中显示出了有希望的结果。

总结

APS 发病机制的最新研究揭示了新的治疗干预领域。已经修订和制定了诊断标准和建议,以提供共识和标准化诊断。

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Aspirin for prevention of preeclampsia in lupus pregnancy.阿司匹林用于预防狼疮妊娠中的子痫前期。
Autoimmune Dis. 2014;2014:920467. doi: 10.1155/2014/920467. Epub 2014 Mar 20.
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Pregnancy morbidity in antiphospholipid syndrome: what is the impact of treatment?抗磷脂综合征中的妊娠并发症:治疗的影响有哪些?
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Antiphospholipid syndrome presenting as pulmonary thrombosis and pancreatitis in a young man.一名年轻男性出现以肺血栓形成和胰腺炎为表现的抗磷脂综合征。
BMJ Case Rep. 2012 Dec 12;2012:bcr2012006348. doi: 10.1136/bcr-2012-006348.