Joëlle Guitard1, Anne-Laure Hebral1, Fadi Fakhouri2, Dominique Joly3, Eric Daugas4, Joseph Rivalan5, Vincent Guigonis6, Françis Ducret7, Claire Presne8, Yves Pirson9, Maryvonne Hourmant2, Jean-Claude Glachant10, Benoit Vendrely11, Olivier Moranne12, Stanislas Faguer1, Dominique Chauveau1. 1. Département de Néphrologie et Transplantation d'organes, CHU Rangueil, Toulouse, France. 2. Service de Néphrologie et Transplantation rénale, CHU Hôtel-Dieu, Nantes, France. 3. Service de Néphrologie, CHU Necker Enfants Malades, AP-HP, Paris, France. 4. Service de Néphrologie et Transplantation, CHU Bichat, AP-HP, Paris, France. 5. Service de Néphrologie, CHU de Pontchaillou, Rennes, France. 6. Service de Néphrologie Pédiatrique, CHU Dupuytren, Limoges, France. 7. Service de Néphrologie, Centre Hospitalier Annecy - Genevois, Annecy, France. 8. Service de Néphrologie, CHU Amiens-Picardie, Amiens, France. 9. Service de Néphrologie, Cliniques Universitaires Saint-Luc, Bruxelles, Belgique. 10. Service de Néphrologie, Centre Hospitalier de Bourg-en-Bresse, Bourg-en-Bresse, France. 11. Service de Néphrologie, CHU Pellegrin, Bordeaux, France. 12. Service de Néphrologie et Transplantation rénale, CHU Pasteur, Nice, France.
Abstract
BACKGROUND: Minimal-change nephrotic syndrome (MCNS) is a common cause of steroid sensitive nephrotic syndrome (NS) with frequent relapse. Although steroids and calcineurin inhibitors (CNIs) are the cornerstone treatments, the use of rituximab (RTX), a monoclonal antibody targeting B cells, is an efficient and safe alternative in childhood. METHODS: Because data from adults remain sparse, we conducted a large retrospective and multicentric study that included 41 adults with MCNS and receiving RTX. RESULTS:Complete (NS remission and withdrawal of all immunosuppressants) and partial (NS remission and withdrawal of at least one immunosuppressants) clinical responses were obtained for 25 and 7 patients, respectively (overall response 78%), including 3 patients that only received RTX and had a complete clinical response. After a follow-up time of 39 months (6-71), relapses occurred in 18 responder patients [56%, median time 18 months (3-36)]. Seventeen of these received a second course of RTX and then had a complete (n = 13) or partial (n = 4) clinical response. From multivariate analysis, on-going mycophenolate mofetil (MMF) therapy at the time of RTX was the only predictive factor for RTX failure [HR = 0.07 95% CI (0.01-0.04), P = 0.003]. Interestingly, nine patients were still in remission at 14 months (3-36) after B-cell recovery. No significant early or late adverse event occurred after RTX therapy. CONCLUSIONS:RTX is safe and effective in adult patients with MCNS and could be an alternative to steroids or CNIs in patients with a long history of relapsing MCNS.
RCT Entities:
BACKGROUND: Minimal-change nephrotic syndrome (MCNS) is a common cause of steroid sensitive nephrotic syndrome (NS) with frequent relapse. Although steroids and calcineurin inhibitors (CNIs) are the cornerstone treatments, the use of rituximab (RTX), a monoclonal antibody targeting B cells, is an efficient and safe alternative in childhood. METHODS: Because data from adults remain sparse, we conducted a large retrospective and multicentric study that included 41 adults with MCNS and receiving RTX. RESULTS: Complete (NS remission and withdrawal of all immunosuppressants) and partial (NS remission and withdrawal of at least one immunosuppressants) clinical responses were obtained for 25 and 7 patients, respectively (overall response 78%), including 3 patients that only received RTX and had a complete clinical response. After a follow-up time of 39 months (6-71), relapses occurred in 18 responder patients [56%, median time 18 months (3-36)]. Seventeen of these received a second course of RTX and then had a complete (n = 13) or partial (n = 4) clinical response. From multivariate analysis, on-going mycophenolate mofetil (MMF) therapy at the time of RTX was the only predictive factor for RTX failure [HR = 0.07 95% CI (0.01-0.04), P = 0.003]. Interestingly, nine patients were still in remission at 14 months (3-36) after B-cell recovery. No significant early or late adverse event occurred after RTX therapy. CONCLUSIONS:RTX is safe and effective in adult patients with MCNS and could be an alternative to steroids or CNIs in patients with a long history of relapsing MCNS.
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