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Am J Kidney Dis. 2018 Jul;72(1):84-92. doi: 10.1053/j.ajkd.2017.11.019. Epub 2018 Feb 9.

Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy.

Author information

1
Department of Nephrology, Centre Hospitalier Universitaire de Montpellier, Montpellier, France.
2
Division of Nephrology, Department of Pediatrics, Centre Hospitalier Universitaire Sainte Justine and University of Montreal, Montréal, Québec, Canada.
3
Department of Nephrology, Hôpital Huriez, Centre Hospitalier Universitaire de Lille, Lille.
4
Department of Pediatric Nephrology, Hospices Civils de Lyon, Bron.
5
Department of Nephrology, Centre Hospitalier Universitaire de Bordeaux, Bordeaux.
6
Department of Pediatric Nephrology, Centre Hospitalier Universitaire Robert Debré.
7
Department of Nephrology, Centre Hospitalier Universitaire Bichat, Paris.
8
Department of Pediatric Nephrology, Centre Hospitalier Universitaire de Toulouse, Toulouse.
9
Department of Nephrology, Hôpital Foch, Suresnes.
10
Department of Pediatric Nephrology, Centre Hospitalier Universitaire de la Timone, Marseille.
11
Department of Nephrology, Centre hospitalier William Morey, Chalon sur Saône.
12
Department of Nephrology, Centre Hospitalier Universitaire Necker, Paris.
13
Department of Nephrology, Hôpitaux du Léman, Léman.
14
Department of Nephrology, Centre Hospitalier Universitaire Henri Mondor, Créteil.
15
Department of Immunology, Centre Hospitalier Universitaire Hôpital Européen Georges Pompidou, Paris.
16
Department of Nephrology and Immunology, Centre Hospitalier Universitaire de Nantes, Nantes, France. Electronic address: fadi.fakhouri@univ-nantes.fr.

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 Québec, 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:

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

PMID:
29429752
DOI:
10.1053/j.ajkd.2017.11.019

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