Khamashta M A, Hughes G R
Lupus Research Unit, Rayne Institute, St. Thomas' Hospital, London, UK.
Curr Opin Rheumatol. 1995 Sep;7(5):389-94. doi: 10.1097/00002281-199509000-00005.
More than a decade has gone by since the detailed clinical description of antiphospholipid syndrome (APS). Thrombosis, the main complication of the syndrome, can affect vessels of all sizes; the consistent histopathologic lesion is a bland thrombus without inflammation. Animal models are providing important new data on clinical and pathogenic aspects of APS. New data on the biology of the so-called cofactor beta 2-glycoprotein I is now available. Clearly, the mode of presentation of the phospholipid antigen appears significant, and beta 2-glycoprotein I may play an important part. Regarding treatment, there is further confirmation that long-term anticoagulation therapy with maintenance of a high international normalized ratio is needed in patients with antiphospholipid antibody-associated thrombosis to prevent recurrences.
自抗磷脂综合征(APS)的详细临床描述以来,已经过去了十多年。血栓形成是该综合征的主要并发症,可影响各种大小的血管;一致的组织病理学病变是无炎症的单纯血栓。动物模型正在为APS的临床和致病方面提供重要的新数据。关于所谓辅因子β2-糖蛋白I的生物学新数据现已可得。显然,磷脂抗原的呈现方式似乎很重要,β2-糖蛋白I可能起重要作用。关于治疗,有进一步证实,抗磷脂抗体相关血栓形成的患者需要长期抗凝治疗并维持高国际标准化比值以预防复发。