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Open AccessReview

A Narrative Review on C3 Glomerulopathy: A Rare Renal Disease

Department of Emergency and Organ Transplantation, Renal Unit, University of Bari, 70124 Bari, Italy
Schena Foundation, European Center for the Study of Renal Diseases, 70010 Valenzano, Italy
Department of Internal Medicine, Division of Nephrology, Dialysis and Transplantation, University of Genoa and IRCCS Ospedale Policlinico San Martino, 16132 Genova, Italy
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2020, 21(2), 525;
Received: 5 December 2019 / Revised: 9 January 2020 / Accepted: 10 January 2020 / Published: 14 January 2020
In April 2012, a group of nephrologists organized a consensus conference in Cambridge (UK) on type II membranoproliferative glomerulonephritis and decided to use a new terminology, “C3 glomerulopathy” (C3 GP). Further knowledge on the complement system and on kidney biopsy contributed toward distinguishing this disease into three subgroups: dense deposit disease (DDD), C3 glomerulonephritis (C3 GN), and the CFHR5 nephropathy. The persistent presence of microhematuria with or without light or heavy proteinuria after an infection episode suggests the potential onset of C3 GP. These nephritides are characterized by abnormal activation of the complement alternative pathway, abnormal deposition of C3 in the glomeruli, and progression of renal damage to end-stage kidney disease. The diagnosis is based on studying the complement system, relative genetics, and kidney biopsies. The treatment gap derives from the absence of a robust understanding of their natural outcome. Therefore, a specific treatment for the different types of C3 GP has not been established. Recommendations have been obtained from case series and observational studies because no randomized clinical trials have been conducted. Current treatment is based on corticosteroids and antiproliferative drugs (cyclophosphamide, mycophenolate mofetil), monoclonal antibodies (rituximab) or complement inhibitors (eculizumab). In some cases, it is suggested to include sessions of plasma exchange. View Full-Text
Keywords: C3 glomerulopathy; Dense deposits disease; C3 glomerulonephritis; CFHR5 nephropathy C3 glomerulopathy; Dense deposits disease; C3 glomerulonephritis; CFHR5 nephropathy
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Schena, F.P.; Esposito, P.; Rossini, M. A Narrative Review on C3 Glomerulopathy: A Rare Renal Disease. Int. J. Mol. Sci. 2020, 21, 525.

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