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BackgroundC3 glomerulopathy associated with monoclonal gammopathy (C3G-MIg) is a rare entity. Herein we analysed the clinical and histologic features of a cohort of C3G-MIg patients.MethodsWe conducted a retrospective, multicentre, observational study. Patients diagnosed with C3G-MIg between 1995 and 2021 were enrolled. All had genetic studies of the alternative complement pathway. The degree of disease activity and chronicity were analysed 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)].ResultsThe study group included 23 patients with a median age 63 of years [interquartile range (IQR) 48–70], and 57% were males. Immunoglobulin G kappa was the most frequent MIg (65%). The diagnosis of C3G-MIg was made in transplanted kidneys in seven patients (30%). Five (22%) patients had C3 nephritic factor and five (22%) had anti-factor H antibodies. One patient carried a pathogenic variant in the CFH gene. During a follow-up of 40 months (IQR 14–69), nine patients (39%) reached KF and these patients had a significantly higher total chronicity score on kidney biopsy. Patients who received clone-targeted therapy had a significantly higher survival compared with other management. Those who achieved haematological response had a significantly higher kidney survival. Outcome was remarkably poor in kidney transplant recipients, with five 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.ConclusionsThe 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 a haematological response are associated with better kidney prognosis.

Introduction: Previous reports have shown very high mortality among hemodialyisis patients. Our goal was to analyze the mortality of patients in the Renal Registry of Patients who remained exclusively on hemodialysis treatment. Methods: The cohort of patients who started treatment in the community of Castilla-La Mancha between 2010 and 2012 and remained on hemodialysis treatment was analysed until the end of 2017. Age, sex, primary kidney disease, vascular access, hemoglobin, Charlson index and serum albumin were included. Results: Mortality rate was 63,4% after 5 years and 76% at the end of the study, with no difference between males and females, and was linked to an older age, urgent onset or in those with acute deterioration of chronic kidney disease, the use of catheters or albumin less than 3.5 g/dl. Conclusions: Mortality in patients who remain on hemodialysis is very high and is associated with non-modifiable factors such as age but also others that we can prevent or treat such as type of vascular access or nutrition status at the beginning of treatment. Resumen: Introducción: Estudios previos han demostrado una elevada mortalidad de los pacientes en tratamiento con hemodiálisis aunque en pocos de ellos se analiza la supervivencia de los que reciben exclusivamente este tratamiento. Nuestro objetivo fue analizar la mortalidad de los pacientes que recibieron tratamiento con hemodiálisis. Métodos: Se analizó la cohorte de pacientes que iniciaron tratamiento sustitutivo entre los años 2010 y 2012 en la comunidad de Castilla-La Mancha y permanecieron en tratamiento con hemodiálisis. Se estudiaron las variables edad, sexo, enfermedad renal primaria, acceso vascular, hemoglobina, índice de Charlson y albúmina sérica al comienzo del tratamiento y se realizó un seguimiento hasta final de 2017. Resultados: La mortalidad fue del 63,4% a los 5 años y del 76% al final del periodo de seguimiento, sin diferencias entre varones y mujeres, y se relacionó con una mayor edad, el comienzo urgente o en aquellos con enfermedad renal reagudizada, la utilización de catéteres o una albúmina inferior a 3,5 g/dl. Conclusiones: La mortalidad en los pacientes que permanecen en diálisis es muy elevada y se asocia a factores no modificables como la edad pero también a otros que podemos prevenir o tratar como el tipo de acceso vascular o el estado de nutrición al comienzo del tratamiento.