系统性红斑狼疮中的血栓性微血管病

Thrombotic microangiopathy in systemic lupus erythematosus

血栓性微血管病(TMA)是一种以微血管血栓形成为特点的累及多器官的临床病理综合征。系统性红斑狼疮(SLE)患者产生抗含Ⅰ型血小板结合蛋白基序的解聚蛋白样金属蛋白酶(ADAMTS13)抗体、补体过度活化、合并感染及使用某些药物时需警惕TMA的发生。SLE合并TMA者病情危重,常规治疗应答差,及早开启血浆置换治疗、合理使用卡普赛珠单抗或依库珠单抗有助于改善患者预后。文章旨在帮助风湿科医生了解SLE-TMA的研究进展,早期识别并及早启动个体化治疗,降低病死率。

Abstrct:Thrombotic microangiopathy(TMA)is a clinicopathologic syndrome characterized by microvascular thrombosis involving multiple organs.Patients with systemic lupus erythematosus(SLE)should be vigilant for the occurrence of TMA,especially for those having anti-ADAMTS13 antibodies,excessive complement activation,infections or using certain drugs.SLE patients with TMA are critically ill and have poor response to routine treatment.Early initiation of plasma exchange therapy and proper use of Caplacizumab or Eculizumab helps to improve prognosis.This article aims to help rheumatologists understand the research progress of SLE combined with TMA,identify and start individualized treatment as early as possible to reduce mortality.