Pediatric Nephrology

, Volume 32, Issue 6, pp 1023–1028 | Cite as

C3 glomerulopathy and eculizumab: a report on four paediatric cases

  • Célia Lebreton
  • Justine Bacchetta
  • Frédérique Dijoud
  • Lucie Bessenay
  • Véronique Fremeaux-Bacchi
  • Anne Laure Sellier-Leclerc
Original Article



Eculizumab may be used to treat C3-glomerulopathy (C3G), a rare but severe glomerular disease.

Diagnosis and Treatment

Patients 1, 2 and 3 were diagnosed with nephritic syndrome with alternative complement pathway activation (low C3, C3Nef-positive) and C3G at the age of 9, 13 and 12 years, respectively. Treatment with eculizumab normalized proteinuria within 1, 2 and 7 months, respectively. Proteinuria relapsed when eculizumab was withdrawn, but the re-introduction of eculizumab normalized proteinuria. Patient 4 was diagnosed with C3G at 9 years of age, with progression to end-stage renal disease within 2 years, followed by a first renal transplantation (R-Tx) with early disease recurrence and graft loss within 39 months. After a second R-Tx, she rapidly presented with biological and histological recurrence: therapy with eculizumab was started, with no effect on proteinuria after 5 months, in a complex clinical setting (i.e. association of C3G recurrence, humoral rejection and BK nephritis). Eculizumab was withdrawn due to multiple viral reactivations, but the re-introduction of the drug a few months later enabled a moderate decrease in proteinuria.


These cases illustrate the efficacy of eculizumab, at least on native kidneys, in paediatric C3G. However, larger international studies are warranted to confirm the benefit and safety of eculizumab therapy.


C3 glomerulonephritis Paediatrics Eculizumab Recurrence post-transplantation Children 



The authors would like to thank Dr Marie-Nathalie Sarda (Department of Biology, Hospices Civils de Lyon) for her help in local complement assessment, and also Dr Moglie Le Quintrec, Pr Eric Rondeau, Pr Chantal Loirat, Pr Fadi Fakhouri and Dr Sophie Chauvet for their expertise during the French multidisciplinary review of the first two cases.

Compliance with ethical standards


The local ethical committee (CPP Lyon Sud Est II) approved this retrospective review of cases (IRB 00009118, session 1/27/2016).

Disclosure of interest

JB received travel grants from Alexion. ALL received travel grants from Alexion and honararia from Alexion (2013, 2014). VFB acts as a scientific advisor for Alexion.


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Copyright information

© IPNA 2017

Authors and Affiliations

  1. 1.Service de Néphrologie Rhumatologie Dermatologie Pédiatriques, Centre de Référence des Maladies Rénales Rares Hôpital Femme Mère EnfantHospices Civils de LyonBronFrance
  2. 2.Faculté de Médecine Lyon EstLyonFrance
  3. 3.Laboratoire d’Anatomopathologie, Groupe Hospitalier EstHospices Civils de LyonBronFrance
  4. 4.Service de PédiatrieCHU Clermont FerrandClermont FerrandFrance
  5. 5.Laboratoire d’Immunologie, Hôpital Georges PompidouAssistance publique–Hôpitaux de Paris (AP-HP)ParisFrance

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