RT Journal Article
T1 Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease.
A1 Caravaca-Fontan, Fernando
A1 Diaz-Encarnacion, Montserrat M
A1 Lucientes, Laura
A1 Cavero, Teresa
A1 Cabello, Virginia
A1 Ariceta, Gema
A1 Quintana, Luis F
A1 Marco, Helena
A1 Barros, Xoana
A1 Ramos, Natalia
A1 Rodriguez-Mendiola, Nuria
A1 Cruz, Sonia
A1 Fernandez-Juarez, Gema
A1 Rodriguez, Adela
A1 Perez de Jose, Ana
A1 Rabasco, Cristina
A1 Rodado, Raquel
A1 Fernandez, Loreto
A1 Perez Gomez, Vanessa
A1 Avila, Ana I
A1 Bravo, Luis
A1 Lumbreras, Javier
A1 Allende, Natalia
A1 Sanchez de la Nieta, Maria Dolores
A1 Rodriguez, Eva
A1 Olea, Teresa
A1 Melgosa, Marta
A1 Huerta, Ana
A1 Miquel, Rosa
A1 Mon, Carmen
A1 Fraga, Gloria
A1 de Lorenzo, Alberto
A1 Draibe, Juliana
A1 Cano-Megias, Marta
A1 Gonzalez, Fayna
A1 Shabaka, Amir
A1 Lopez-Rubio, Maria Esperanza
A1 Fenollosa, Maria Angeles
A1 Martin-Penagos, Luis
A1 Da Silva, Iara
A1 Alonso Titos, Juana
A1 Rodriguez de Cordoba, Santiago
A1 Goicoechea de Jorge, Elena
A1 Praga, Manuel
K1 Alternative complement pathway
K1 C3 glomerulopathy
K1 Mycophenolate mofetil
AB C3 glomerulopathy is a complement-mediated disease arising from abnormalities in complement genes and/or antibodies against complement components. Previous studies showed that treatment with corticosteroids plus mycophenolate mofetil (MMF) was associated with improved outcomes, although the genetic profile of these patients was not systematically analyzed. This study aims to analyze the main determinants of disease progression and response to this therapeutic regimen. We conducted a retrospective, multicenter, observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. Patients diagnosed with C3 glomerulopathy (n=81) or dense deposit disease (n=16) between January 1995 and March 2018 were enrolled. Multivariable and propensity score matching analyses were used to evaluate the association of clinical and genetic factors with response to treatment with corticosteroids and MMF as measured by proportion of patients with disease remission and kidney survival (status free of kidney failure). The study group comprised 97 patients (84% C3 glomerulopathy, 16% dense deposit disease). Forty-two patients were treated with corticosteroids plus MMF, and this treatment was associated with a higher rate of remission and lower probability of kidney failure (79% and 14%, respectively) compared with patients treated with other immunosuppressives (24% and 59%, respectively), or ecluzimab (33% and 67%, respectively), or conservative management (18% and 65%, respectively). The therapeutic superiority of corticosteroids plus MMF was observed both in patients with complement abnormalities and with autoantibodies. However, patients with pathogenic variants in complement genes only achieved partial remission, whereas complete remissions were common among patients with autoantibody-mediated forms. The main determinant of no remission was baseline proteinuria. Relapses occurred after treatment discontinuation in 33% of the patients who had achieved remission with corticosteroids plus MMF, and a longer treatment length of MMF was associated with a lower risk of relapse. The beneficial response to corticosteroids plus MMF treatment in C3 glomerulopathy appears independent of the pathogenic drivers analyzed in this study.
PB American Society of Nephrology
YR 2020
FD 2020-08-19
LK http://hdl.handle.net/10668/16118
UL http://hdl.handle.net/10668/16118
LA en
NO Caravaca-Fontán F, Díaz-Encarnación MM, Lucientes L, Cavero T, Cabello V, Ariceta G, et al. Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease. Clin J Am Soc Nephrol. 2020 Sep 7;15(9):1287-1298
DS RISalud
RD Apr 12, 2025