C3 Glomerulopathy and post-infectious glomerulonephritis define a disease spectrum

Pediatr Nephrol. 2016 Nov;31(11):2079-86. doi: 10.1007/s00467-015-3311-3. Epub 2016 Mar 23.

Abstract

Background: Post-infectious glomerulonephritis (PIGN) usually follows a benign course, but few children have an atypical, severe presentation, and these exceptional cases have been linked to the dysregulation of the complement alternative pathway (CAP). There is a considerable overlap in the histopathological features of PIGN and C3 glomerulopathy (C3G), which is also associated with CAP dysregulation but has a poorer outcome. We hypothesized that PIGN and C3G define a disease spectrum, and that in the past there may be some children with C3G who were misclassified with PIGN before C3G was described as a separate disease entity.

Methods: Children with PIGN (n = 33) diagnosed between 1985 and 2010 who underwent a renal biopsy due to their unusual course were reviewed and of them, 8 were reclassified into C3G based on the current classification criteria. Outcome was based on the degree of proteinuria, C3 level, and renal function at follow-up.

Results: Sixteen (72.7%) children with typical PIGN recovered completely as compared to only 2 (25%) with C3G. Of note, children with "typical" PIGN had a more severe disease course at onset; however, the outcome at last follow up was favorable.

Conclusions: Our results support the hypothesis that PIGN and C3G form a disease spectrum and have different long-term clinical implications and management strategies.

Keywords: C3 glomerulonephritis; C3 glomerulopathy; Complement alternative pathway; Dense deposit disease; Eculizumab; Post-infectious glomerulonephritis.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Biopsy
  • Child
  • Child, Preschool
  • Communicable Diseases / complications*
  • Complement C3 / analysis*
  • Complement C3 / metabolism
  • Complement Pathway, Alternative*
  • Female
  • Glomerulonephritis / blood
  • Glomerulonephritis / etiology
  • Glomerulonephritis / metabolism*
  • Glomerulonephritis / pathology
  • Glomerulonephritis, Membranoproliferative / blood
  • Glomerulonephritis, Membranoproliferative / metabolism*
  • Glomerulonephritis, Membranoproliferative / pathology
  • Humans
  • Kidney Glomerulus / pathology
  • Longitudinal Studies
  • Male
  • Proteinuria / urine

Substances

  • Complement C3