Eculizumab for treatment of rapidly progressive C3 glomerulopathy.

Abstract

C3 glomerulopathy (C3G) is a prototypic complement-mediated kidney disease. Rapidly progressive forms of C3G usually respond poorly to conventional treatments. We report on the efficacy of the terminal complement inhibitor eculizumab in 3 adult patients with rapidly progressive C3G. In all 3 patients, serum creatinine levels had increased by >50% in the 2 months preceding initiation of eculizumab treatment despite the use of conventional immunosuppressive drugs and/or plasma exchanges in 2 of these individuals. Of note, 2 patients had long-standing nephrotic syndrome. Kidney biopsy performed prior to eculizumab treatment disclosed marked glomerular inflammatory changes and increased C5b-9 deposition in all patients. Eculizumab use was associated with significant improvement in kidney function, with estimated glomerular filtration rates of patients increasing 22 to 38 mL/min/1.73 m(2). Eculizumab use also was associated with remission of nephrotic syndrome in the 2 affected patients, an effect observed as early as one week after treatment initiation. Repeat kidney biopsy disclosed regression of glomerular inflammatory changes and decreases in glomerular staining for C5b-9 in all patients. These results warrant further assessment of eculizumab for treatment of rapidly progressive forms of C3G with markedly increased glomerular C5b-9 deposits.

• Authors: Moglie Le Quintrec, Arnaud Lionet, Christine Kandel, Franck Bourdon, Viviane Gnemmi, Magali Colombat, Jean-Michel Goujon, Véronique Frémeaux-Bacchi, Fadi Fakhouri
• Journal: American journal of kidney diseases : the official journal of the National Kidney Foundation (Am J Kidney Dis) Vol. 65 Issue 3 Pg. 484-9 (Mar 2015) ISSN: 1523-6838 [Electronic] United States
• PMID: 25530108 (Publication Type: Case Reports, Journal Article, Multicenter Study)
• Copyright: Copyright © 2015 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

Chemical References

• Antibodies, Monoclonal, Humanized
• Complement C3
• eculizumab

Topics

• Adult
• Antibodies, Monoclonal, Humanized (administration & dosage)
• Complement C3 (analysis, metabolism)
• Female
• Glomerulonephritis, Membranoproliferative (diagnosis, drug therapy, metabolism)
• Humans
• Infusions, Intravenous
• Male
• Middle Aged
• Treatment Outcome