儿童系统性红斑狼疮并发血栓性微血管病的临床特点和早期识别  

Clinical characteristics and early recognition of thrombotic microangiopathy in children with systemic lupus erythematosus

作　　者：殷蕾[1] 茅幼英[1] 周征宇[1] 沈小钰[1] 黄华 丁飞 金燕樑 周纬[1] Yin Lei;Mao Youying;Zhou Zhengyu;Shen Xiaoyu;Huang Hua;Ding Fei;Jin Yanliang;Zhou Wei(Department of Nephrology,Shanghai Children′s Medical Center,School of Medicine,Shanghai Jiaotong University,Shanghai 200127,China;Department of Rheumatology,Shanghai Children′s Medical Center,School of Medicine,Shanghai Jiaotong University,Shanghai 200127,China)

机构地区：[1]上海交通大学医学院附属上海儿童医学中心肾脏科,200127 [2]上海交通大学医学院附属上海儿童医学中心风湿科,200127

出　　处：《中国小儿急救医学》2021年第10期868-873,共6页Chinese Pediatric Emergency Medicine

摘　　要：目的分析儿童系统性红斑狼疮(systemic lupus erythematosus,SLE)并发血栓性微血管病(thrombotic microangiopathy,TMA)的临床特点及其早期识别。方法回顾性总结上海交通大学医学院附属上海儿童医学中心2005年12月至2020年10月收治的14例SLE并发TMA患儿的临床资料。结果并发TMA的患儿占同期SLE住院患儿的5.65%(14/248),占同期狼疮性肾炎患儿的7.87%(14/178)。其中男女比例4∶10,除1例男孩的发病年龄为6岁,其余13例年龄在11~18岁。患儿SLE疾病活动度评分在14~31分。在发生TMA的病程中,11例接受了肾活检,肾脏病理类型是Ⅳ或Ⅳ+Ⅴ型,8例肾脏病理提示有TMA病变。14例患儿在入院后3 d至2个月内被诊断TMA,在入院时只有6例同时存在贫血和血小板减少,另外8例只有中度贫血。所有患儿均存在明显低补体血症,特别是未经治疗的SLE初发病例,血清C3<0.17 g/L,C4<0.07 g/L,均有不同程度的肾小球滤过率下降。随访时间为0.2~11.3年(中位时间为2.6年),经治疗后6例完全缓解;5例部分缓解;1例在第4次血浆置换过程中发生猝死;2例病情恶化。结论儿童SLE并发TMA者多处于疾病重度活动期,肾脏病理类型以Ⅳ型狼疮性肾炎为主;对于有贫血的SLE患儿,不论是否有血小板降低,都要特别关注血清补体水平,若严重降低,要密切监测肾小球滤过率和查找外周血涂片有无裂红细胞,以便于及早发现TMA。Objective To analyze the clinical characteristics of children diagnosed with systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)for early recognition.Methods We retrospectively reviewed the clinical records of 14 SLE patients with TMA hospitalized at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine from December 2005 to October 2020.Results The incidence of TMA was 5.65%(14/248)of the hospitalized patients with SLE and 7.87%(14/178)of the hospitalized patients with lupus nephritis.Four patients were boys while ten patients were girls.One boy was six years old and other 13 patients were from 11 to 18 years old.Their SLEDAI scores ranged from 14 to 31,and all of them were severe activity.Renal biopsy of 11 patients during TMA course all revealed lupus nephritis(typeⅣ,n=8;typeⅣ+Ⅴ,n=3).These 14 SLE children were diagnosed with TMA within 3 days to 2 months after admission.At the beginning of the hospitalization,only six patients had both anemia and thrombocytopenia,while eight patients only had moderate anemia.All of the patients had obvious hypocomplementemia.Especially in the patients with first onset of SLE without treatment,their serum levels of C3 were less than 0.17 g/L and C4 were less than 0.07 g/L.Moreover,glomerular filtration rates of these patients were lower than that in normal range.The follow-up time were 0.2-11.3 years(median time was 2.6 years).After treatment,six patients obtained complete remission,and five patients obtained partial remission.One patient had sudden death during the 4th plasmapheresis,and the other two patients deteriorated.Conclusion Children with SLE and TMA are mostly in severe disease activity,and renal pathology is typeⅣlupus nephritis.The SLE children with anemia should be paid special attention to the level of serum complement whether they have thrombocytopenia or not.If the level of serum complements decrease obviously,glomerular filtration rates should be monitored closely and schistocytes should be s

关 键 词：系统性红斑狼疮 血栓性微血管病 低补体血症 儿童 

分 类 号：R725.4[医药卫生—儿科] R725.9[医药卫生—临床医学]