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Journal of Nephrology

, Volume 31, Issue 4, pp 503–510 | Cite as

Dominant C3 glomerulopathy: new roles for an old actor in renal pathology

  • Nicola Pirozzi
  • Antonella Stoppacciaro
  • Paolo Menè
Review

Abstract

Recently, a number of reports have described dominant C3 deposits in renal biopsies of patients with infection-related glomerulonephritis (GN). While acute post-infectious GN and membranoproliferative GN are commonly characterized by immune deposits containing C3 and/or C4, the absence of immunoglobulin (Ig) and/or immune complexes at light or electron microscopy is a rather unusual observation. Dominant C3 deposition is believed to result from the alternative pathway of complement activation via the C3bBb “tickover” convertase. The actual occurrence of C3 glomerulopathy could be underestimated, since infection-related GN often quickly subsides without the need for a renal biopsy. A more thorough understanding of the pathways that lead to complement assembly and deposition within the kidney is needed to support a new classification of complement-related lesions, including entities such as dense deposit disease, (atypical) hemolytic-uremic syndrome, dominant C1q, CFHR5, C4d, and C3 glomerulopathies. We will briefly review recent work in this area, focusing on GN with selective complement C3 deposits.

Keywords

Glomerulonephritis Complement C3 Renal biopsy Immune deposits 

Notes

Compliance with ethical standards

Informed consent

Informed consent was obtained from all individual participants included in the study (renal biopsies performed for diagnostic purpose). No identifying information about participants is available in the article.

Ethical approval

For this type of retrospective study (review of the literature) formal consent is not required.

Conflict of interest

On behalf of all Authors, the corresponding Author states that there is no conflict of interest.

Research involving human participants and/or animals

This review article does not contain any studies with human participants or animals performed by any of the Authors.

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Copyright information

© Italian Society of Nephrology 2017

Authors and Affiliations

  1. 1.Department of Clinical and Molecular MedicineUniversity of Rome “La Sapienza”RomeItaly
  2. 2.Chair and Division of NephrologySant’Andrea University HospitalRomeItaly
  3. 3.Division of PathologySant’Andrea University HospitalRomeItaly
  4. 4.UOC NefrologiaA.O. Sant’AndreaRomeItaly

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