Abstract
Background
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).
Methods
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.
Results
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.
Conclusions
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.
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Acknowledgments
The authors thank M. Tanji, T. Sato, and K. Honzumi for their assistance.
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The study was carried out under the auspices of the Committee for Human Experiments at the Fukushima Medical University School (Institutional Review Board approval number 1583). Informed consent was obtained from all patients or their parents.
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We have no conflicts of interests affecting the present study.
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Kawasaki, Y., Kanno, S., Ono, A. et al. Differences in clinical findings, pathology, and outcomes between C3 glomerulonephritis and membranoproliferative glomerulonephritis. Pediatr Nephrol 31, 1091–1099 (2016). https://doi.org/10.1007/s00467-015-3307-z
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DOI: https://doi.org/10.1007/s00467-015-3307-z