Introduction
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.
Methods
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]).
Results
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.
Conclusions
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.
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