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The Role of Rituximab in Primary Focal Segmental Glomerulosclerosis of the Adult

Authors :
Tedesco, M
Mescia, F
Pisani, I
Allinovi, M
Casazza, G
Del Vecchio, L
Santostefano, M
Cirillo, L
Ferrario, F
Esposito, C
Esposito, P
Santoro, D
Lazzarin, R
Rossi, G
Fiaccadori, E
Ferrantelli, A
Sinico, R
Cozzolino, M
Gallieni, M
Cirami, L
Scolari, F
Vaglio, A
Alberici, F
Affatato, S
Caroti, L
Mancini, E
Semeraro, L
Siligato, R
Cassia, M
Napodano, P
Calatroni, M
Distratis, C
Campo, A
Tedesco, Martina
Mescia, Federica
Pisani, Isabella
Allinovi, Marco
Casazza, Giovanni
Del Vecchio, Lucia
Santostefano, Marisa
Cirillo, Luigi
Ferrario, Francesca
Esposito, Ciro
Esposito, Pasquale
Santoro, Domenico
Lazzarin, Roberta
Rossi, Giovanni Maria
Fiaccadori, Enrico
Ferrantelli, Angelo
Sinico, Renato Alberto
Cozzolino, Mario
Gallieni, Maurizio
Cirami, Lino
Scolari, Francesco
Vaglio, Augusto
Alberici, Federico
Affatato, Stefania
Caroti, Leonardo
Mancini, Elena
Semeraro, Luca
Siligato, Rossella
Cassia, Matthias Arnaldo
Napodano, Pietro
Calatroni, Marta
Distratis, Cosimo
Campo, Andrea
Tedesco, M
Mescia, F
Pisani, I
Allinovi, M
Casazza, G
Del Vecchio, L
Santostefano, M
Cirillo, L
Ferrario, F
Esposito, C
Esposito, P
Santoro, D
Lazzarin, R
Rossi, G
Fiaccadori, E
Ferrantelli, A
Sinico, R
Cozzolino, M
Gallieni, M
Cirami, L
Scolari, F
Vaglio, A
Alberici, F
Affatato, S
Caroti, L
Mancini, E
Semeraro, L
Siligato, R
Cassia, M
Napodano, P
Calatroni, M
Distratis, C
Campo, A
Tedesco, Martina
Mescia, Federica
Pisani, Isabella
Allinovi, Marco
Casazza, Giovanni
Del Vecchio, Lucia
Santostefano, Marisa
Cirillo, Luigi
Ferrario, Francesca
Esposito, Ciro
Esposito, Pasquale
Santoro, Domenico
Lazzarin, Roberta
Rossi, Giovanni Maria
Fiaccadori, Enrico
Ferrantelli, Angelo
Sinico, Renato Alberto
Cozzolino, Mario
Gallieni, Maurizio
Cirami, Lino
Scolari, Francesco
Vaglio, Augusto
Alberici, Federico
Affatato, Stefania
Caroti, Leonardo
Mancini, Elena
Semeraro, Luca
Siligato, Rossella
Cassia, Matthias Arnaldo
Napodano, Pietro
Calatroni, Marta
Distratis, Cosimo
Campo, Andrea
Publication Year :
2022

Abstract

Introduction: Primary focal segmental glomerular sclerosis (FSGS) is a rare, likely immune-mediated disease. Rituximab (RTX) may play a role in management, although data in adults are scanty. Methods: We collected cases of RTX-treated primary FSGS within the Italian Society of Nephrology Immunopathology Working Group and explored response rate (24-hour proteinuria <3.5 g and <50% compared with baseline, stable estimated glomerular filtration rate). Results: A total of 31 patients were followed for at least 12 months; further follow-up (median 17 months, interquartile range [IQR] 15–33.5) was available for 11. At first RTX administration, median creatinine and 24-hour proteinuria were 1.17 mg/dl (IQR 0.83–1.62) and 5.2 g (IQR 3.3–8.81), respectively. Response rate at 3, 6, and 12 months was 39%, 52%, and 42%, respectively. In the first 12 months, creatinine level remained stable whereas proteinuria and serum albumin level improved, with an increase in the proportion of patients tapering other immunosuppressants. There were 6 patients who were retreated with RTX within 12 months, either for proteinuria increase or refractory disease; only the 2 responders to the first RTX course experienced a further response. At univariate analysis, 6-month response was more frequent in steroid-dependent patients (odds ratio [OR] 7.7 [95% CI 1.16–52.17]) and those with proteinuria <5 g/24 h (OR 8.25 [1.45–46.86]). During long-term follow-up, 4 of 5 responders at 12 months maintained a sustained response, either without further immunosuppression (2 of 4) or with pre-emptive RTX (2 of 4); 1 relapsed and responded to RTX retreatment. Conclusion: RTX may be an option in primary FSGS, especially in steroid-dependent patients, with 24-hour proteinuria <5 g and previously responders to RTX. Optimal long-term management for responders is unclear, with some patients experiencing sustained remission and others requiring RTX retreatment, either preemptive or after rising proteinuria

Details

Database :
OAIster
Notes :
ELETTRONICO, English
Publication Type :
Electronic Resource
Accession number :
edsoai.on1334334865
Document Type :
Electronic Resource