伊库珠单抗治疗儿童系统性红斑狼疮合并血栓性微血管病的2例经验分享及文献复习

Eculizumab for treatment of thrombotic microangiopathy secondary to systemic lupus erythematosus in children:two cases report and literature review

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种累及全身多系统、器官的严重自身免疫性疾病。血栓性微血管病(thrombotic microangiopathy,TMA)是一类临床上少见但可导致机体多个脏器障碍甚至危及生命的临床综合征。临床上SLE合并TMA的患者较为少见,儿童更为罕见,合并TMA的SLE患者死亡率显著增高。该文报道2例儿童SLE合并TMA使用伊库珠单抗治疗后病情缓解的病例,并探讨儿童SLE合并TMA的临床诊断、表现和治疗特点,为其诊治提供临床依据。

Systemic lupus erythematosus(SLE)is a serious autoimmune disease that affects multiple systems and organs throughout the body.Thrombotic microangiopathy(TMA)is a rare clinical syndrome that can lead to multiple organ dysfunction and even threaten life.Clinically,SLE patients with TMA are relatively rare,especially in children,but the mortality of SLE patients with TMA is significantly increased.The article reports 2 cases of successful remission of SLE complicated with TMA after treatment with eculizumab,and discusses the diagnosis,clinical manifestations and treatment of SLE complicated with TMA in children,so as to provide clinical basis for the diagnosis and treatment of SLE complicated with TMA in children.