系统性红斑狼疮并发血栓性微血管病  

Systemic lupus erythematosus complicated with thrombotic microangiopathy

作　　者：杨杏林 张上珠 徐东 曾小峰 YANG Xing-lin;ZHANG Shang-zhu;XU Dong;ZENG Xiao-feng(Department of Rheumatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Key Laboratory of Rheumatology and Clinical Immunology,Ministry of Education,Beijing 100730,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院风湿免疫科,风湿免疫病学教育部重点实验室,北京100730

出　　处：《中华临床免疫和变态反应杂志》2018年第5期545-551,共7页Chinese Journal of Allergy & Clinical Immunology

基　　金：国家重点研发计划(2016YFC0901500、2016YFC0901501)~~

摘　　要：血栓性微血管病(TMA)是一种多器官受累的临床急症,其特点为微血管病性溶血性贫血及血小板减少。有少数系统性红斑狼疮(SLE)患者可并发TMA,其病因多种多样,包括继发血栓性血小板减少性紫癜、继发补体介导的TMA、药物诱导的TMA、病毒感染导致的TMA等。此外,SLE患者可继发抗磷脂抗体综合征,可并发系统性硬化症以及恶性高血压等疾病,这些疾病也可通过各自独特的病理生理机制导致TMA。SLE并发TMA患者病情危重,预后极差,不同病因治疗原则亦不相同,迅速识别病因并积极治疗非常重要。Thrombotic microangiopathy(TMA)describes a clinical emergency involving multiple organ systems,characterized by microangiopathic hemolytic anemia and thrombocytopenia.Systemic lupus erythematosus(SLE)complicated with TMA is not uncommon,with a complex pathophysiology including secondary thrombotic thrombocytopenic purpura,secondary complement-mediated TMA,drug-induced thrombotic microangiopathie,virus infection-induced TMA,and so on.In addition,TMA can be caused through unique pathophysiological mechanisms in antiphospholipid syndrome,systemic sclerosis,and malignant hypertension,which could be seen in SLE patients.SLE patients complicated with TMA usually present with severe manifestations and had poor prognosis.Different therapeutic approaches are required for each clinical situation.Identifying the causes quickly and selecting appropriate treatments are of crucial importance.

关 键 词：血栓性微血管病 系统性红斑狼疮 微血管病性溶血性贫血 血小板减少 

分 类 号：R593.241[医药卫生—内科学] R543[医药卫生—临床医学]