灾难性抗磷脂抗体综合征的诊断与治疗

灾难性抗磷脂抗体综合征发生于抗磷脂抗体阳性的患者，多继发于原发性抗磷脂抗体综合征、系统性红斑狼疮等。起病多有明确诱因，如感染、创伤、抗凝异常等。临床表现为小血管血栓形成导致多器官功能障碍及系统性炎症反应综合征。近年通过早期诊断，采取有效治疗，包括抗凝、糖皮质激素、血浆置换、静脉丙种球蛋白注射等，使死亡率显著下降。

Early Diagnosis and Treatment of Catastrophic Antiphospholipid Syndrome

The catastrophic antiphospholipid syndrome （CAPS） develops rapidly following an identifiable triggering factor （e.g. infection, trauma, inadequate coagulation） in antiphospholipid antibody positive patients. It is most frequently encountered in patients with a primary antiphospholipid syndrome or systemic lupus erythematosus （SLE）. It manifests mainly with small vessel thrombosis of organs, manifestations of the systemic inflammatory response syndrome （SIRS）. The mortality is high. Early diagnosis and effective therapies, including full parenteral anticoagulation, corticosteroids, plasma exchanges and IV globulins, could significantly reduce the mortality rate.