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C3 nephritic factor associated with C3 glomerulopathy in children

Camille Nicolas1, Vincent Vuiblet, Veronique Baudouin

  • 1Department of Pediatrics, Nephrology unit, CHU de Reims, Reims, France.

Pediatric Nephrology (Berlin, Germany)
|September 27, 2013
PubMed

Insights

C3 glomerulopathy (C3G) in children associated with C3 nephritic factor (C3NeF) shows varied symptoms and outcomes. Anti-proteinuric treatments may help manage the condition, but immunosuppressive therapy effectiveness is uncertain.

Area of Science:

  • Nephrology
  • Complement System Biology
  • Pediatric Nephrology

Background:

  • C3 glomerulopathy (C3G) involves abnormal complement alternative pathway activity and C3 deposition in kidneys.
  • C3 nephritic factor (C3NeF) is present in half of C3G patients, encompassing dense deposit disease (DDD) and C3 glomerulonephritis (C3GN).

Purpose of the Study:

  • To investigate pediatric cases of C3G specifically associated with C3NeF.
  • To analyze the clinical presentation, renal histology, and outcomes in children with C3NeF-associated C3G.

Main Methods:

  • Retrospective review of 18 pediatric C3G cases with C3NeF.
  • Analysis of clinical data, kidney biopsy findings (C3GN, DDD), and complement levels (C3, sC5b9).
  • Exclusion of patients with identified mutations in CFH, CFI, and MCP genes.

Main Results:

  • Presentations included recurrent hematuria, nephrotic syndrome, acute kidney injury, and post-infectious glomerulonephritis.
  • Low C3 levels were observed in 12 patients; kidney biopsies revealed C3GN (10) and DDD (8).
  • Relapse occurred in 7 patients; long-term outcomes included dialysis (2), persistent proteinuria (11), and variable treatment responses.

Conclusions:

  • C3NeF-associated C3G in children presents heterogeneously with diverse clinical courses.
  • Anti-proteinuric agents show potential for disease control, even post-nephrotic syndrome.
  • The efficacy of immunosuppressive treatments like steroids requires further investigation.
Abstract

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