Abstract
C3 glomerulonephritis (C3GN) is a rare form of glomerulopathy that is characterized by predominant C3 deposits. Eculizumab, a humanized monoclonal C5 antibody, has recently emerged as a treatment option for C3GN. We report a C3GN patient successfully treated with eculizumab. A 5-year-old boy who presented with proteinuria, hematuria, high ASO titers, and low C3 levels was initially diagnosed with post-streptococcal GN. His first kidney biopsy confirmed this diagnosis, but complement investigations identified three alternative pathway dysregulation factors: C3 nephritic factor, complement factor I heterozygous mutation (I398L), and anti-factor H autoantibodies (4,500 AU/ml). A second biopsy performed 11 months after initial presentation (nephrotic range proteinuria) showed a C3GN suggestive of isolated C3 deposits. Despite the use of intensive immunosuppressive therapy (rituximab, corticosteroids, mycophenolate), nephrotic-range proteinuria persisted and a third kidney biopsy showed the same C3GN pattern with more endocapillary proliferation. The serum C5b-9 level was elevated. Eculizumab was initiated and resulted in a significant decline of proteinuria (5.3 to 1.3 g/day) and an improvement in pathologic features. A transient i...Continue Reading
References
Mar 1, 1995·The Journal of Clinical Investigation·K HøgåsenM Harboe
Dec 6, 2002·The American Journal of Pathology·Guido A HegasyPeter F Zipfel
Oct 14, 2004·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Gareth JonesAine Burns
Oct 31, 2008·Clinical Journal of the American Society of Nephrology : CJASN·Samih H NasrVivette D D'Agati
Dec 18, 2009·Kidney International·Frank BienaimeVeronique Fremeaux-Bacchi
Apr 28, 2010·Clinical Nephrology·M YuanX Ding
Mar 19, 2011·Clinical Journal of the American Society of Nephrology : CJASN·Sanjeev SethiRichard J H Smith
Mar 10, 2012·Clinical Journal of the American Society of Nephrology : CJASN·Andrew S BombackGerald B Appel
Mar 23, 2012·The New England Journal of Medicine·Sanjeev Sethi, Fernando C Fervenza
Mar 23, 2012·The New England Journal of Medicine·Erica DainaGiuseppe Remuzzi
Mar 23, 2012·The New England Journal of Medicine·Marina VivarelliFrancesco Emma
Mar 30, 2012·Kidney International·Aude ServaisVéronique Frémeaux-Bacchi
Jun 9, 2012·Journal of the American Society of Nephrology : JASN·Leal C HerlitzVivette D D'Agati
Dec 14, 2012·Kidney International·Sanjeev SethiRichard J H Smith
May 22, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sevgi GurkanRichard J Smith
Sep 27, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Camille NicolasChristine Pietrement
Nov 1, 2013·Kidney International·Matthew C PickeringH Terence Cook
Jan 11, 2014·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Caroline Rousset-RouvièreMichel Tsimaratos
Citations
Aug 8, 2015·Clinical Kidney Journal·Laura Rodriguez-Osorio, Alberto Ortiz
May 9, 2016·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Lindsay S Keir, Craig B Langman
Jul 28, 2016·Nature Reviews. Nephrology·Marie-Agnes Dragon DureyArvind Bagga
Aug 17, 2016·Clinical Journal of the American Society of Nephrology : CJASN·Shveta S MotwaniUNKNOWN American Society of Nephrology Onco-Nephrology Forum
Oct 30, 2016·The American Journal of Pathology·Maria G DetsikaElias A Lianos
Sep 30, 2016·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Hassib ChehadeManuel Pascual
Mar 30, 2018·Case Reports in Nephrology and Dialysis·Ravneet BajwaArif Asif
Jan 10, 2018·World Journal of Transplantation·Fedaey AbbasAhmed Halawa
Jan 14, 2018·BMC Nephrology·Thomas WelteElke Neumann-Haefelin
Nov 6, 2018·Nephrology·Nika KojcTanja Kersnik Levart
Oct 6, 2017·Clinical Kidney Journal·Vicky Brocklebank, David Kavanagh
Jun 3, 2017·BioMed Research International·Michael Rudnicki
May 10, 2019·Frontiers in Immunology·Fernando CorvilloMargarita López-Trascasa
Oct 9, 2016·Diagnostic Pathology·Tanja Kersnik LevartNika Kojc
Mar 2, 2021·Frontiers in Immunology·Jacob J E KoopmanCees van Kooten