伴C1q沉积的儿童原发性膜性肾病的临床特点及预后

Clinical features and prognosis of primary membranous nephropathy in children with C1q deposition

目的分析伴C1q沉积的儿童原发性膜性肾病(PMN)的临床特点及预后。方法回顾性分析2005年7月至2013年9月东部战区总医院经肾活检确诊的177例PMN患儿的临床资料,根据肾脏病理免疫荧光C1q染色是否阳性分为C1q沉积组和非C1q沉积组。比较2组患儿的临床病理特征、治疗反应及远期预后。结果177例儿童PMN包括男98例,女79例。中位年龄为192.0个月。经过(52.4±35.6)个月的随访,7例(4.0%)进展至终末期肾病(ESRD),14例(7.9%)进展至ESRD或肾功能不全。C1q沉积组血IgG水平较非C1q沉积组高[(5.10±2.51)g/L比(4.34±2.10)g/L,t=2.110,P=0.036],肾小球C4沉积阳性率较非C1q沉积组高(34.7%比2.9%,χ^(2)=32.567,P<0.001)。Kaplan-Meier生存分析显示,2组间ESRD的累积肾脏生存率(P=0.561)和累积缓解率(P=0.291)的差异均无统计学意义。Logistic回归分析显示,C1q沉积与治疗反应无关(P=0.587)。单因素COX回归分析显示,性别(男)(HR=8.578,95%CI:1.120~65.689,P=0.039)和尿蛋白未缓解(HR=0.053,95%CI:0.017~0.171,P<0.001)为肾功能不全的危险因素。多因素分析结果显示,尿蛋白未缓解(HR=21.858,95%CI:5.595~85.387,P<0.001)和C1q沉积(HR=0.116,95%CI:0.023~0.584,P=0.009)为肾功能不全的独立危险因素。结论C1q沉积是预示肾功能不全的独立危险因素。补体激活的经典途径在部分PMN患儿中被激活,且可能在介导肾损伤中发挥重要作用。

Objective To analyse the clinical and prognosis of C1q deposition in children with primary membranous nephropathy(PMN).Methods A retrospective analysis was conducted in 177 children with PMN who were diagnosed by renal biopsy in the Eastern Theater Cornmand General Hospital from July 2005 to September 2013.Patients were divided into C1q deposit group and C1q non-deposit group according to the immunofluorescence staining of C1q.Clinical and pathological characteristics,treatment response,and long-term renal prognosis were compared between the 2 groups.Results A total of 177 pediatric patients with PMN were included,involving 98 boys and 79 girls with a median age of 192.0 months.During an follow-up of(52.4±35.6)months,7 cases(4.0%)progressed end-stage renal disease(ESRD),and 14 cases(7.9%)developed ESRD or renal dysfunction.The blood IgG level of C1q deposit group was higher than that of C1q non-deposit group[(5.10±2.51)g/L vs.(4.34±2.10)g/L,t=2.110,P=0.036].The frequency of glomerular C4 deposits in C1q deposit group was significantly higher than that of C1q non-deposit group(34.7%vs.2.9%,χ^(2)=32.567,P<0.001).The Kaplan-Meier survival analysis showed that there were no differences in cumulative renal survival rate of ESRD(P=0.561)and cumulative incidence rate of remission(P=0.291)between groups.The Logistic regression analysis demonstrated that C1q deposition was not correlated with treatment responses(P=0.587).Univariate COX regression analysis demonstrated that the male gender(HR=8.578,95%CI:1.120-65.689,P=0.039)and no remission(HR=0.053,95%CI:0.017-0.171,P<0.001)were risk factors for renal dysfunction in children with PMN.Multivariate COX regression analysis reveled that no remission(HR=21.858,95%CI:5.595-85.387,P<0.001)and C1q deposition(HR=0.116,95%CI:0.023-0.584,P=0.009)were independent risk factors for renal dysfunction in children with PMN.Conclusions C1q deposition was an independent risk factor for renal dysfunction in children with PMN.The classical pathway does occur in some PMN patients,which plays an essential role in mediating kidney injury.