Differences in clinical findings, pathology, and outcomes between C3 glomerulonephritis and membranoproliferative glomerulonephritis
Differences in clinical findings, pathology, and outcomes between C3 glomerulonephritis and membranoproliferative glomerulonephritis
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DOI：
10.1007/s00467-015-3307-z
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发表时间：
2016-07-01
影响因子：
3
通讯作者：
Hosoya, Mitsuaki
中科院分区：
文献类型：
--
作者：
Kawasaki, Yukihiko;Kanno, Syuto;Hosoya, Mitsuaki
为了明确补体成分C3肾小球肾炎(C3GN)的临床表现，我们回顾分析了C3GN与免疫复合物介导膜增生性肾小球肾炎(IC-MPGN)在临床病理表现及预后方面的差异。将患者分为两组，第一组19例诊断为IC-MPGN，第二组18例诊断为C3GN。对两组的临床表现和预后进行了调查。第二组的13名患者通过强制性的年度学校尿液异常筛查得到确认。2组患者肉眼血尿发生率及低C4值发生率均低于1组。在第二次肾活检时，第二组患者的尿蛋白排泄率、血尿发生率、低C3值频率以及系膜增生、肾小球硬化和间质纤维化评分均高于第一组患者。在最近的随访检查中，第二组患者被归类为无反应或终末期肾病的患者数量高于第一组患者。我们的结果表明，C3GN患者的治疗反应和预后比IC介导的MPGN患者更差。因此，在有关治疗选择和预后的临床背景下，将特发性MPGN分类为C3GN或IC-MPGN可能是有用的。此外，C3GN的长期随访也是必要的。
To clarify the clinical manifestations of pediatric complement component C3 glomerulonephritis (C3GN), we retrospectively evaluated differences in the clinicopathological findings and prognosis between C3GN and immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN).Thirty-seven patients diagnosed with "idiopathic MPGN" were enrolled in this retrospective study. The patients were divided into two groups, with Group 1 consisting of 19 patients diagnosed with IC-MPGN and Group 2 consisting of 18 patients diagnosed with C3GN. The clinical findings and the prognosis were investigated for both groups.Thirteen patients in Group 2 were identified by mandatory annual school screening for urinary abnormalities. The incidence of macro-hematuria and the frequency of low serum C4 values were lower in Group 2 patients than in Group 1 patients. At the time of the second renal biopsy, urinary protein excretion, incidence of hematuria, frequency of low serum C3 values, and scores for mesangial proliferation, glomerular sclerosis, and interstitial fibrosis were higher in Group 2 patients than in Group 1 patients. At the most recent follow-up examination, the number of patients categorized as non-responding or with end-stage renal disease was higher in Group 2 patients than in Group 1 patients.Our results suggest that the treatment response and prognosis of patients with C3GN are worse than those of patients with IC-mediated MPGN. Therefore, in the clinical context regarding treatment options and prognosis, it may be useful to classify idiopathic MPGN as C3GN or IC-MPGN. In addition, long-term follow-up of C3GN is necessary.