摘要
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.
作者
章玲霞
王晶晶
金艳艳
刘飞
傅海东
陈冰蓉
毛建华
Zhang Lingxia;Wang Jingjing;Jin Yanyan;Liu Fei;Fu Haidong;Chen Bingrong;Mao Jianhua(Department of Nephrology,Children Hospital of Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Key Laboratory of Traditional Chinese Medicine for Non-infectious Chronic Diseases in Children of Zhejiang Province,Hangzhou 310053,China;Department of Pediatrics,the First People's Hospital of Yongkang City,Zhejiang Province,Yongkang 321300,China)
出处
《中华肾脏病杂志》
CAS
CSCD
北大核心
2023年第12期942-946,共5页
Chinese Journal of Nephrology
基金
国家重点研发计划(2021YFC2702002)
浙江省重点研发计划(2021C03079)。
关键词
儿童
红斑狼疮
系统性
血栓性微血管病
伊库珠单抗
治疗
Child
Lupus erythematosus,systemic
Thrombotic microangiopathies
Eculizumab
Treatment