Blockade of C5 in Severe Acute Postinfectious Glomerulonephritis Associated With Anti-Factor H Autoantibody.
Activation of the complement cascade plays an important role in the pathogenesis of postinfectious glomerulonephritis. We report successful terminal complement pathway blockade using an anti-C5 monoclonal antibody (eculizumab) in an 8-year-old child with severe acute postinfectious glomerulonephritis requiring hemodialysis. The child presented with clinical, serologic, and histopathologic criteria for diffuse crescentic postinfectious glomerulonephritis. Complement measurements showed low C3 and C4 levels, with increased SC5b-9 titers. The presence of a transient anti-factor H autoantibody was also identified. Eculizumab (600mg, 2 doses at a 1-week interval) was administered, with a striking recovery of kidney function. There were no additional hemodialysis sessions needed after the first dose of eculizumab, and glomerular filtration rate measured using inulin clearance at 12 months of follow-up was within the normal range (92mL/min/1.73m2). Prompt terminal complement blockade may have improved the outcome in this case of severe acute postinfectious glomerulonephritis.
Pediatric Nephrology Unit, Service of Pediatrics, University Hospital of Lausanne (CHUV), Lausanne, Switzerland. Electronic address: hassib.chehade@chuv.ch.
Hassib
H
Chehade
Pathology Institute, University Hospital of Lausanne (CHUV), Lausanne, Switzerland.
Samuel
S
Rotman
Laboratory of Immunology and Biology, Hôpital Européen Georges-Pompidou, AP-HP, Paris, France.
Véronique
V
Frémeaux-Bacchi
Service of Immunology, University Hospital of Lausanne (CHUV), Lausanne, Switzerland.
Vincent
V
Aubert
Service of Immunology, University Hospital of Lausanne (CHUV), Lausanne, Switzerland.
Salima
S
Sadallah
Pediatric Nephrology Unit, Service of Pediatrics, University Hospital of Lausanne (CHUV), Lausanne, Switzerland.
Loukia
L
Sifaki
Service of Pediatric Nephrology, Necker Children's Hospital, Paris, France.
Rémi
R
Salomon
Transplantation Center, University Hospital of Lausanne (CHUV), Lausanne, Switzerland. Electronic address: manuel.pascual@chuv.ch.
Manuel
M
Pascual
D000208:Acute Disease; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D001323:Autoantibodies; D002648:Child; D003176:Complement C3 / Q000037:antagonists & inhibitors*; D017242:Complement Factor H / Q000276:immunology*; D005921:Glomerulonephritis / Q000188:drug therapy* / Q000276:immunology* / Q000382:microbiology; D006801:Humans; D008297:Male; D012720:Severity of Illness Index
D061067:Antibodies, Monoclonal, Humanized; D001323:Autoantibodies; D003176:Complement C3; D017242:Complement Factor H; C481642:eculizumab
CFH; Postinfectious glomerulonephritis (PIGN); acute kidney injury; anti-C5 monoclonal antibody; anti–factor H autoantibody; case report; complement; crescentic PIGN; eculizumab; kidney function; pediatric; renal biopsy; terminal complement pathway blockade