Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy
Details
Serval ID
serval:BIB_2A577D725E68
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy
Journal
Am J Kidney Dis
ISSN
1523-6838 (Electronic)
ISSN-L
0272-6386
Publication state
Published
Issued date
07/2018
Volume
72
Number
1
Pages
84-92
Language
english
Notes
Le Quintrec, Moglie
Lapeyraque, Anne-Laure
Lionet, Arnaud
Sellier-Leclerc, Anne-Laure
Delmas, Yahsou
Baudouin, Veronique
Daugas, Eric
Decramer, Stephane
Tricot, Leila
Cailliez, Mathilde
Dubot, Philippe
Servais, Aude
Mourey-Epron, Catherine
Pourcine, Franck
Loirat, Chantal
Fremeaux-Bacchi, Veronique
Fakhouri, Fadi
eng
Am J Kidney Dis. 2018 Jul;72(1):84-92. doi: 10.1053/j.ajkd.2017.11.019. Epub 2018 Feb 9.
Lapeyraque, Anne-Laure
Lionet, Arnaud
Sellier-Leclerc, Anne-Laure
Delmas, Yahsou
Baudouin, Veronique
Daugas, Eric
Decramer, Stephane
Tricot, Leila
Cailliez, Mathilde
Dubot, Philippe
Servais, Aude
Mourey-Epron, Catherine
Pourcine, Franck
Loirat, Chantal
Fremeaux-Bacchi, Veronique
Fakhouri, Fadi
eng
Am J Kidney Dis. 2018 Jul;72(1):84-92. doi: 10.1053/j.ajkd.2017.11.019. Epub 2018 Feb 9.
Abstract
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Quebec, Canada. OUTCOMES: Global or partial clinical renal response. MEASUREMENTS: Evolution of serum creatinine and proteinuria values. RESULTS: 26 patients (13 children/adolescents) were included. 22 (85%) patients had received steroids, plasma exchange, or immunosuppressive therapy before eculizumab, and 3 of them had rapid progression of their kidney disease despite treatment. At the initiation of eculizumab therapy, 11 (42%) patients had chronic kidney disease, 7 (27%) had rapidly progressive disease, and 3 (12%) required dialysis. After eculizumab treatment (median duration, 14 months), 6 (23%) patients had a global clinical response; 6 (23%), a partial clinical response; and 14 (54%), no response. Compared with those who had a partial clinical or no response, patients who had a global clinical response had lower estimated glomerular filtration rates, a more rapidly progressive course, and more extracapillary proliferation on kidney biopsy. Age, extent of renal fibrosis, frequency of nephrotic syndrome, low serum C3 and C3 nephritic factor and elevated soluble C5b-9 concentrations, or complement gene variants did not differ between responders and nonresponders. LIMITATIONS: Retrospective design without a control group, relatively small number of cases, inclusion of pediatric and adult cases. CONCLUSIONS: Eculizumab appears to be a potential treatment for patients with crescentic rapidly progressive C3 glomerulopathy. Its benefit in patients with non-rapidly progressing forms seems to be limited.
Keywords
Adolescent, Adult, Aged, Antibodies, Monoclonal, Humanized/*therapeutic use, Child, Complement C3/*metabolism, Female, Follow-Up Studies, Glomerulonephritis, Membranoproliferative/*blood/diagnosis/*drug therapy, Humans, Male, Middle Aged, Retrospective Studies, Treatment Outcome, Young Adult, *C3 glomerulopathy (C3G), *C3 nephritic factor (C3Nef), *anti-C5 monoclonal antibody, *case series, *clinical response, *complement, *eculizumab, *kidney biopsy, *membranoproliferative glomerulonephritis (MPGN), *pediatric, *proteinuria, *rapidly progressive glomerulonephritis
Pubmed
Create date
01/03/2022 10:18
Last modification date
02/03/2022 6:35