ESPN 50th Annual Meeting

ESPN 2017

Response Of A Child With Familial Dysregulation Of The Complement System To Anti-C5 Antibody


C3 glomerulopathy is a new entity of a heterogeneous group of glomerular diseases associated with acquired or genetic abnormalities of complement alternative pathway (AP) components. It is characterized by predominant C3 deposits in the mesangium and along the glomerular basement membrane (GBM). Based on Electron Microscopy, C3 Nephritis was previously classified as Type III Membranoproliferative Glomerulonephritis. Advances in the understanding of processes involved in the pathogenesis through immunoflouresence classified MPGN as being mediated by immune complexes or  by complement dysregulation that leads to persistent activation of the complement AP. As well Classification that is based upon the pathogenic process helps to direct the clinical evaluation and to provide disease-specific treatments. C3 glomerulopathy may present with haematuria, proteinuria or renal failure. 

Material and methods:

We report the clinical progress of a child with a progressive glomerulonephritis through her first decade of life, whose renal biopsy confirmed Proliferative glomerulonephritis with endocapillary and Membranoproliferative patterns, Mesangial and subendothelial deposits with occasional subepithelial deposits on electron microscopy.  Immunoflouresence profile was consistent with C3 related glomerulonephritis. Assessment of the complement panel revealed low C3, CFB & CFH levelS and increased activity of the alternative complement system. Genetic study revealed a familial homozygous miss ensue mutation of CFH. 


The optimal treatment of C3 glomerulopathy remains unknown. It is currently based on the use of angiotensin-converting-enzyme inhibitors (ACEI) and angiotensin II-receptor blockers (ARB), sometimes associated with immunosuppressive therapy.  Plasma infusion and plasma exchange therapy was used by different authors. We also report patient’s response to the humanized anti-C5 antibody. 


A renal biopsy report that denotes C3 glomerulopathy should prompt investigation of the complement system. Identifying patients with dysregulation of the complement system is mandatory as they may benefit from therapeutic use of the complement inhibitor.