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Work on this study was supported by the Instituto de Salud Carlos III/Fondo Europeo de Desarrollo Regional (ISCIII/FEDER; grant PI16/01685 and PI19/1624) and Red de Investigacion Renal (RedInRen; RD12/0021/0029 to MP) and theAutonomous Region of Madrid (S2017/BMD-3673 to MP), E.G.d.J. is supported by the Spanish 'Ministerio de Ciencia, Innovacion y Universidades-FEDER' (RTI2018-095955-B-100) and Autonomous Region ofMadrid (S2017/BMD-3673). None of the funders had any role in the study design, data collection, analyses, reporting or the decision to submit for publication.

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C3 glomerulopathy associated with monoclonal gammopathy: impact of chronic histologic lesions and beneficial effects of clone-targeted therapies

Publicated to:Nephrology Dialysis Transplantation. 37 (11): 2128-2137 - 2022-10-19 37(11), DOI: 10.1093/ndt/gfab302

Authors: Caravaca-Fontán, F; Lucientes, L; Serra, N; Cavero, T; Rodado, R; Ramos, N; Gonzalez, F; Shabaka, A; Cabello, V; Huerta, A; Pampa-Saico, S; Gutiérrez, E; Quintana, LF; López-Rubio, ME; Draibe, J; Titos, JA; Fernández-Juárez, G; De Jorge, EG; Praga, M

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Abstract

C3 glomerulopathy associated with monoclonal gammopathy (C3G-MIg) is a rare entity. Herein, we analyzed the clinical and histologic features of a cohort of C3G-MIg patients.Retrospective, multicenter, observational study. Patients diagnosed with C3G-MIg between 1995-2021 were enrolled. All had genetic studies of the alternative complement pathway. The degree of disease activity and chronicity was analyzed using the C3G histologic index. Descriptive statistics and propensity-score matching (PSM) analysis were used to evaluate the main outcome of the study (kidney failure [KF]).The study group included 23 patients: median age 63 years (IQR 48-70), 57% males. IgG kappa was the most frequent MIg (65%). The diagnosis of C3G-MIg was made in transplanted kidneys in 7 patients (30%). Five (22%) had C3 nephritic factor and 5 (22%) anti-factor H antibodies. One patient carried a pathogenic variant in CFH gene.During a follow-up of 40 months (IQR 14-69), 9 patients (39%) reached KF, and these patients had a significant higher total chronicity score in kidney biopsy. Patients who received clone-targeted therapy had a significant higher survival compared to other management. Those who achieved hematological response had a significant higher kidney survival. Outcome was remarkably poor in kidney transplant recipients, 5 of them (71%) reaching KF.By PSM (adjusting for age, kidney function, proteinuria and chronicity score), no significant differences were observed in kidney survival between C3G patients with/without MIg.The C3G histologic index can be used in patients with C3G-MIg to predict kidney prognosis, with higher chronicity scores being associated with worse outcomes. Clone-targeted therapies and the development of hematological response, are associated with better kidney prognosis.© The Author(s) 2021. Published by Oxford University Press on behalf of the ERA.

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