Skip to main content


Log in

Evolution of immunoglobulin deposition in C3-dominant membranoproliferative glomerulopathy

  • Brief Report
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript



Complement 3 glomerulopathy (C3GN) is a newly proposed subcategory of glomerular disease with features including membranoproliferative glomerulonephritis (MPGN), C3-dominant immunofluorescent staining without appreciable immunoglobulin deposition, and electron-dense deposits. Aberrations of alternative complement pathway (AP) have been found in many C3GN patients.


A 13-year-old boy presented with edema in association with an upper respiratory infection. Studies demonstrated nephrotic syndrome with hematuria and markedly low C3 and C4. Initial renal biopsy showed MPGN with strong C3 and immunoglobulin deposition. The patient partially responded to immunosuppression. Follow-up biopsies at 10 months and 3 years demonstrated MPGN with strong C3, with little to no immunoglobulin deposition. Based on this and elevated SC5b-9, treatment was changed to eculizumab with further decrease in proteinuria.


Serial biopsies illustrated marked variability in immunoglobulin deposition in MPGN with persistently strong C3 deposition. Whether this evolution was related to the course of disease or to therapeutic intervention, the pathologic progression documented in this series of biopsies challenges the newly proposed subcategories of MPGN.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1


  1. Fakhouri F, Frémeaux-Bacchi V, Noël LH, Cook HT, Pickering MC (2010) C3 glomerulopathy: a new classification. Nat Rev Nephrol 6:494–499

    Article  PubMed  CAS  Google Scholar 

  2. Sethi S, Fervenza FC (2012) Membranoproliferative glomerulonephritis–a new look at an old entity. N Engl J Med 366:1119–1131

    Article  PubMed  CAS  Google Scholar 

  3. Sethi S, Nester CM, Smith RJ (2012) Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion. Kidney Int 81:434–441

    Article  PubMed  Google Scholar 

  4. Barbour TD, Pickering MC, Cook HT (2013) Recent insights into C3 glomerulopathy. Nephrol Dial Transplant 28:1685–1693

    Google Scholar 

  5. Larsen CP, Walker PD (2013) Redefining C3 glomerulopathy: ‘C3 only’ is a bridge too far. Kidney Int 83:331-332. Author replies: Sethi S (2013) Kidney Int 83:332; Servais A, Noel LH, Lesavre P, Frémeaux-Bacchi V (2013) Kidney Int 83:332–333

    Google Scholar 

  6. Fervenza FC, Sethi S, Glassock RJ (2012) Idiopathic membranoproliferative glomerulonephritis: does it exist? Nephrol Dial Transplant 27:4288–4294

    Article  PubMed  CAS  Google Scholar 

  7. Uchida T, Oda T, Watanabe A, Izumi T, Higashi K, Kushiyama T, Miura S, Kumagai H (2011) Clinical and histologic resolution of poststreptococcal glomerulonephritis with large subendothelial deposits and kidney failure. Am J Kidney Dis 58:113–117

    Article  PubMed  Google Scholar 

  8. D’Agati VD, Bomback AS (2012) C3 glomerulopathy: what’s in a name? Kidney Int 82:379–381

    Article  PubMed  Google Scholar 

  9. Sethi S, Fervenza FC, Zhang Y, Zand L, Meyer NC, Borsa N, Nasr SH, Smith RJ (2013) Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int 83:293–299

    Article  PubMed  CAS  Google Scholar 

  10. Sandhu G, Bansal A, Ranade A, Jones J, Cortell S, Markowitz GS (2012) C3 glomerulopathy masquerading as acute postinfectious glomerulonephritis. Am J Kidney Dis 60:1039–1043

    Article  PubMed  Google Scholar 

  11. Servais A, Noël LH, Roumenina LT, Le Quintrec M, Ngo S, Dragon-Durey MA, Macher MA, Zuber J, Karras A, Provot F, Moulin B, Grünfeld JP, Niaudet P, Lesavre P, Frémeaux-Bacchi V (2012) Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 82:454–464

    Article  PubMed  CAS  Google Scholar 

  12. Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, Theis JD, Dogan A, Smith RJ (2012) C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int 82:465–473

    Article  PubMed  CAS  Google Scholar 

  13. Herlitz LC, Bomback AS, Markowitz GS, Stokes MB, Smith RN, Colvin RB, Appel GB, D’Agati VD (2012) Pathology after eculizumab in dense deposit disease and C3 GN. J Am Soc Nephrol 23:1229–1237

    Article  PubMed  CAS  Google Scholar 

Download references


The authors would like to thank Alex Bolinder for expert technical work on Fig. 1m, and Drs. Donald Houghton and Jose Rueda for critical reading of the manuscript.

Author information

Authors and Affiliations


Corresponding author

Correspondence to Megan L. Troxell.

Electronic supplementary materials

Below is the link to the electronic supplementary material.

Supplementary Table 1a

Laboratory studies (DOC 42 kb)

Supplementary Table 1b

Plasma complement studies (DOC 42 kb)

Supplementary Table 2

Biopsy findings (DOCX 13.8 kb)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kerns, E., Rozansky, D. & Troxell, M.L. Evolution of immunoglobulin deposition in C3-dominant membranoproliferative glomerulopathy. Pediatr Nephrol 28, 2227–2231 (2013).

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: