摘要
目的探讨儿童狼疮性肾炎(LN)合并血栓性微血管病(TMA)的临床和病理特征及预后。方法病例对照研究。回顾性分析2008年1月至2023年1月在徐州医科大学附属徐州市儿童医院经肾活检明确诊断为LN患儿的临床和病理资料,其中46例LN患儿合并TMA(LN-TMA组)。根据年龄、性别、和病理类型进行匹配,选取92例(1∶2)无TMA的LN患儿作为对照组(LN组)。采用Kaplan-Meier法评估LN患儿的总体和肾脏存活率,采用Cox回归模型分析进展为终末期肾脏病(ESRD)的风险因素。结果TMA与血肌酐(Scr)、血清C3、抗C1q抗体(a-C1q)、估算肾小球滤过滤(eGFR)、毛细血管内增生、纤维素样坏死和肾组织C1q沉积呈中度相关(均r>0.5)。血清a-C1q≥20 U/mL(HR=8.724,95%CI:0.976~16.114,P=0.026)和eGFR≤60 mL/(min·1.73 m^(2))(HR=12.213,95%CI:1.147~25.048,P=0.038)是LN儿童发生TMA的独立危险因素。肾小球硬化(HR=7.228,95%CI:0.186~22.358,P=0.016)、TMA(HR=11.387,95%CI:3.426~42.554,P=0.009)和eGFR≤60 mL/(min·1.73 m^(2))(HR=3.116,95%CI:0.592~10.064,P=0.030)是LN患儿进展为ESRD的独立危险因素。LN-TMA组5年和10年肾脏生存率低于LN组(97.44%比98.28%,80.90%比87.27%)(χ^(2)=4.918,P=0.027)。结论LN-TMA患儿病情危重,预后差。TMA是LN儿童进展为ESRD的独立危险因素,其发生机制可能与补体激活相关。
Objective To investigate the clinical and pathological characteristics and prognosis of children with lupus nephritis(LN)and thrombotic microangiopathy(TMA).Methods In this retrospective case-control study,clinical and pathological data of LN children confirmed by renal biopsy from January 2008 to January 2023 in Xuzhou Children′s Hospital,Xuzhou Medical University were analyzed.There were 46 LN children complicated with TMA(LN-TMA group).With matched age,sex and pathology,92 LN children(1∶2)without TMA were selected as the control group(LN group).The Kaplan-Meier method was used to evaluate the overall and renal survival rates of children with LN,and the Cox regression model was used to analyze the risk factors for the progression to end-stage renal disease(ESRD).Results TMA was moderately associated with serum creatinine,serum C3,anti-C1q antibody(a-C1q),estimated glomerular filtration rate(eGFR),endocapillary proliferation,fibrinoid necrosis,and renal C1q deposition(all r>0.5).Serum a-C1q≥20 U/mL(HR=8.724,95%CI:0.976-16.114,P=0.026)and eGFR≤60 mL/(min·1.73 m^(2))(HR=12.213,95%CI:1.147-25.048,P=0.038)were independent risk factors for TMA in children with LN.Glomerular sclerosis(HR=7.228,95%CI:0.186-22.358,P=0.016),TMA(HR=11.387,95%CI:3.426-42.554,P=0.009)and eGFR≤60 mL/(min·1.73 m^(2))(HR=3.116,95%CI:0.592-10.064,P=0.030)were independent risk factors for developing ESRD in LN children.The 5-year and 10-year renal survival rates in the LN-TMA group were lower than those in the LN group(97.44%vs.98.28%,80.90%vs.87.27%,χ^(2)=4.918,P=0.027).Conclusions Children with LN-TMA present with severe symptoms and poor prognosis.TMA is an independent risk factor for progression to ESRD in children with LN,and the mechanism may be related to complement activation.
作者
刘海鹏
刘小微
赵英荣
朱峰
Liu Haipeng;Liu Xiaowei;Zhao Yingrong;Zhu Feng(Department of Emergency,Xuzhou Children′s Hospital,Xuzhou Medical University,Xuzhou 221000,China;Intensive Care Unit,Xuzhou Infectious Disease Hospital,Xuzhou 221000,China)
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2024年第3期203-208,共6页
Chinese Journal of Applied Clinical Pediatrics
关键词
狼疮性肾炎
血栓性微血管病
儿童
补体激活
Lupus nephritis
Thrombotic microangiopathy
Child
Complement activation
