急性链球菌感染后肾小球肾炎与C3肾小球病的关系

摘要

目的 探讨急性链球菌感染后肾小球肾炎(APSGN)与C3肾小球病的关系,提高对C3肾小球病认识.方法 选择2006年1月至2015年9月北京大学第一医院儿科收治的入院初诊考虑为APSGN并行肾脏活检的患儿为研究对象,依据最终诊断分为APSGN组和非APSGN组.对其临床资料进行回顾性统计分析,并结合随访情况,比较2组患儿临床表现、肾脏病理、补体变化情况和转归等.结果 28例患儿中,最终诊断为APSGN 19例;非APSGN组共9例,其中C3肾小球病7例[致密物沉积病1例,C3肾小球肾炎6例],血栓性微血管病1例,系膜增生性IgA肾病1例.APSGN组和C3肾小球病亚组患儿发病时的临床症状、肾脏活检组织光镜和免疫荧光表现差异均无统计学意义(P均＞0.05).与C3肾小球病亚组[1例(14.3％)]相比,APSGN组13例(81.2％)患儿补体C3水平在病程8周内恢复;病程1～3年时APSGN组患儿尿常规多可恢复正常,而C3肾小球病亚组仍多有不同程度血尿、蛋白尿,2组间差异具有统计学意义(P＜0.05).1例C3肾小球病患儿有补体旁路途径调节异常的相关实验室证据和致密物沉积病典型电镜表现.结论 部分初始诊断为APSGN的患儿临床表现不典型,注意与C3肾小球病鉴别.需进一步监测其临床表现、尿常规及血C3水平变化,必要时重复肾活检和完善补体旁路途径相关检查,以尽早明确病因、对因治疗和改善预后.%Objective To explore the relationship between C3 glomerulopathy and acute post-streptococcal glomerulonephritis (APSGN),and improve the recognition of C3 glomerulopathy.Methods Clinically diagnosed APSGN patients who were admitted to the Department of Pediatrics of Peking University First Hospital and completed percutaneous renal biopsy were retrospectively collected from January 2006 to September 2015.These patients were divided into APSGN group and non-APSGN group according to the final diagnosis.Clinical data and follow-up survey of these patients were collected and analyzed.Clinical manifestations,renal pathology,change of complement levels,prognosis and so on were compared between the 2 groups.Results Of 28 patients enrolled in this study,19 patients were assigned to APSGN group according to the final diagnosis.The non-APSGN group contained 9 cases,including 7 patients with C3 glomerulopathy(1 case with dense deposit disease and 6 cases with C3 glomerulonephritis),1 patient with thrombotic microangiopathy and 1 patient with mesangial proliferative IgA nephropathy.At the early stage of disease,no significant difference was observed in the clinical manifestation,and the pathology features on light microscopy and immunofluorescence between APSGN group and C3 glomerulopathy subgroup(all P ＞ 0.05).Compared with C3 glomerulopathy subgroup [1 case (14.3 ％)],the C3 levels of 13 cases (81.2％) of APSGN patients could return to normal within 8 weeks after presentation.The urinary abnormalities in the APSGN patients disappeared within 1 to 3 years,while the C3 glomerulopathy subgroup patients had different degrees of persistent hematuria and proteinuria.These 2 phenomena were of statistical difference (all P ＜ 0.05).One patient with C3 glomerulopathy also had a defect in the regulation of the alternative pathway of complement,and a typical electron-dense appearance of dense deposit disease on electron microscopy.Conclusions The clinical manifestations of some initially diagnosed APSGN patients can be atypical.Sufficient attention must be paid to the differential diagnosis of APSGN and C3 glomerulopathy.For earlier diagnosis,more appropriate treatment and better prognosis of these patients,further monitoring of the clinical manifestations,urine analysis and serum C3 levels is required.Repeat renal biopsy and complement investigations on C3 glomerulopathy should be performed as needed.