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Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children
Kidney International Reports
  • Amrit Kirpalani, Hospital for Sick Children University of Toronto
  • Natasha Jawa, Hospital for Sick Children University of Toronto
  • William E. Smoyer, Research Institute at Nationwide Childrens Hospital
  • Christoph Licht, Hospital for Sick Children University of Toronto
  • Gina Marie Barletta
  • Sharon Bartosh
  • Neal Blatt
  • Tom Blydt-Hansen
  • Patrick Brophy
  • Lawrence Copelovitch
  • Brad Dixon
  • Anne Durkan
  • Matthew Eison
  • Larry Greenbaum
  • Guillermo Hidalgo
  • Deborah Jones
  • Mini Michael
  • John Sanders
  • Donald Weaver
  • Amy Wilson
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Introduction: The reclassification of membranoproliferative glomerulonephritis (MPGN) into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) has provided insights into 2 distinct diseases. Although outcomes in adults are poor in both diseases, the pediatric literature is scarce and limited to small, single-center cohorts. Methods: We conducted a retrospective analysis of 165 pediatric patients across 17 hospitals to compare outcomes between children with IC-MPGN and C3G. Results: Forty-two percent of patients initially diagnosed with MPGN were reclassified as C3G after a review of renal biopsy reports. There was a trend toward higher serum creatinine levels in patients with C3G compared with IC-MPGN both at diagnosis (mean 168.9 [range 45.4–292.4] vs. 93.7 [range 70.7–116.6] μmol/l, P = 0.25) and after a mean follow-up time of 4 years (mean 145.0 (range −8.1 to 298.1) vs 99.1 (range 46.3–151.9) μmol/l, P = 0.47), although the estimated glomerular filtration rate (eGFR) was not significantly different. Steroid treatment was associated with a significant improvement in eGFR versus no steroids in C3G (mean +43.0 (range 12.9–73.0) vs. −3.0 (range −23.1 to 17.2) ml/min per 1.73 m2, P = 0.02) but not in IC-MPGN. Overall kidney function was preserved in both groups although hypertension remained prevalent in 42.5% of the cohort at the last follow-up, and the urine protein/creatinine ratio remained elevated (mean 253.8 [range 91.9–415.7] mg/mmol). Conclusion: This large pediatric IC-MPGN/C3G cohort revealed nearly half of the patients were misclassified, and there may be a trend toward worse renal prognosis in C3G although they may have greater steroid responsiveness. The overall prognosis appears to be more favorable than in adults; however, persistent hypertension and proteinuria suggest suboptimal disease control.

Citation Information
Amrit Kirpalani, Natasha Jawa, William E. Smoyer, Christoph Licht, et al.. "Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children" Kidney International Reports Vol. 5 Iss. 12 (2020) p. 2313 - 2324
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