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抗磷脂综合征

The antiphospholipid syndrome.

作者信息

Hughes G R, Khamashta M A

机构信息

Lupus Arthritis Research Unit, Rayne Institute, London.

出版信息

J R Coll Physicians Lond. 1994 Jul-Aug;28(4):301-4.

Abstract

Ten years ago we described a group of patients with a combination of clinical features associated with the presence of antiphospholipid antibodies. These features included a tendency to both arterial and venous thrombosis, livedo reticularis, recurrent abortions and occasional thrombocytopenia. This striking clinical constellation was originally named the anticardiolipin syndrome and is now more appropriately called the antiphospholipid syndrome (APS). Although our early studies were centered on systemic lupus erythematosus in which up to a third of patients demonstrated features of the syndrome, it was clear even a decade ago that the APS would increasingly become the domain of neurologists and cardiovascular physicians. The consensus is that antiphospholipid antibodies have a pathogenetic role in the vasculopathy of the APS, but the mechanisms are still unknown. The establishment of good animal models for the APS is the best opportunity to develop rational and more targeted therapies. In our experience, treatments directed against the secondary thrombotic event have proved more successful than those directed against the underlying immunological abnormality.

摘要

十年前,我们描述了一组具有与抗磷脂抗体存在相关的临床特征组合的患者。这些特征包括动静脉血栓形成倾向、网状青斑、反复流产以及偶尔出现的血小板减少症。这一显著的临床症候群最初被命名为抗心磷脂综合征,现在更恰当地称为抗磷脂综合征(APS)。尽管我们早期的研究集中在系统性红斑狼疮,其中多达三分之一的患者表现出该综合征的特征,但即使在十年前就很明显,APS将越来越多地成为神经科医生和心血管内科医生的领域。目前的共识是,抗磷脂抗体在APS的血管病变中具有致病作用,但其机制仍不清楚。建立良好的APS动物模型是开发合理且更具针对性治疗方法的最佳机会。根据我们的经验,针对继发性血栓形成事件的治疗已被证明比针对潜在免疫异常的治疗更为成功。

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