BACKGROUND: Rituximab (RTX) is a promising option for treating childhood-onset steroid-dependent (SDNS), frequently relapsing (FRNS), and steroid-resistant (SRNS) nephrotic syndrome. METHODS: We retrospectively surveyed RTX treatment for these conditions to evaluate its indications, efficacy and adverse events. Questionnaires were sent to 141 hospitals in Japan. RESULTS: Seventy-four patients (52 SDNS; 3 FRNS; 19 SRNS) were treated with RTX because of resistance to various immunosuppressive agents. Most patients received a single administration of RTX (85%). Forty-one of 53 SDNS/FRNS (77%) and 5 of 17 SRNS (29%) patients successfully discontinued prednisolone (16 SDNS/FRNS and 6 SRNS achieved their first discontinuation since onset), and 17 out of 53 SDNS/FRNS patients (31%) discontinued cyclosporine. However, 28 of the 53 patients (51%) relapsed. Although immunosuppressive agents did not extend B cell depletion, relapses were significantly less if immunosuppressive agents were continued after RTX (P = 0.006; hazard ratio = 0.2). Among the SRNS patients, complete (n = 6) and partial remission (n = 6) were achieved. No life-threatening adverse events were experienced. CONCLUSIONS: Although this was a multi-center survey where treatment of nephrotic syndrome varied between centers, the steroid-sparing effect of RTX in SDNS/FRNS was excellent. If single administration of RTX is chosen, continuation of immunosuppressive agents is recommended for prevention of relapse.
BACKGROUND: Rituximab (RTX) is a promising option for treating childhood-onset steroid-dependent (SDNS), frequently relapsing (FRNS), and steroid-resistant (SRNS) nephrotic syndrome. METHODS: We retrospectively surveyed RTX treatment for these conditions to evaluate its indications, efficacy and adverse events. Questionnaires were sent to 141 hospitals in Japan. RESULTS: Seventy-four patients (52 SDNS; 3 FRNS; 19 SRNS) were treated with RTX because of resistance to various immunosuppressive agents. Most patients received a single administration of RTX (85%). Forty-one of 53 SDNS/FRNS (77%) and 5 of 17 SRNS (29%) patients successfully discontinued prednisolone (16 SDNS/FRNS and 6 SRNS achieved their first discontinuation since onset), and 17 out of 53 SDNS/FRNS patients (31%) discontinued cyclosporine. However, 28 of the 53 patients (51%) relapsed. Although immunosuppressive agents did not extend B cell depletion, relapses were significantly less if immunosuppressive agents were continued after RTX (P = 0.006; hazard ratio = 0.2). Among the SRNS patients, complete (n = 6) and partial remission (n = 6) were achieved. No life-threatening adverse events were experienced. CONCLUSIONS: Although this was a multi-center survey where treatment of nephrotic syndrome varied between centers, the steroid-sparing effect of RTX in SDNS/FRNS was excellent. If single administration of RTX is chosen, continuation of immunosuppressive agents is recommended for prevention of relapse.
Authors: Markus J Kemper; Jutta Gellermann; Sandra Habbig; Rafael T Krmar; Katalin Dittrich; Therese Jungraithmayr; Lars Pape; Ludwig Patzer; Heiko Billing; Lutz Weber; Martin Pohl; Katrin Rosenthal; Anne Rosahl; Dirk E Mueller-Wiefel; Jörg Dötsch Journal: Nephrol Dial Transplant Date: 2011-11-09 Impact factor: 5.992
Authors: D Kumar; S Gourishankar; T Mueller; S Cockfield; J Weinkauf; D Vethanayagam; A Humar Journal: Transpl Infect Dis Date: 2008-09-18 Impact factor: 2.228
Authors: Daniela S Ardelean; Tanja Gonska; Shannon Wires; Ernest Cutz; Anne Griffiths; Elizabeth Harvey; Shirley M L Tse; Susanne M Benseler Journal: Pediatrics Date: 2010-06-21 Impact factor: 7.124
Authors: Kenneth R Carson; Andrew M Evens; Elizabeth A Richey; Thomas M Habermann; Daniele Focosi; John F Seymour; Jacob Laubach; Susie D Bawn; Leo I Gordon; Jane N Winter; Richard R Furman; Julie M Vose; Andrew D Zelenetz; Ronac Mamtani; Dennis W Raisch; Gary W Dorshimer; Steven T Rosen; Kenji Muro; Numa R Gottardi-Littell; Robert L Talley; Oliver Sartor; David Green; Eugene O Major; Charles L Bennett Journal: Blood Date: 2009-03-05 Impact factor: 22.113
Authors: Gabriel Cara-Fuentes; John A Kairalla; Takuji Ishimoto; Christopher Rivard; Richard J Johnson; Eduardo H Garin Journal: Pediatr Nephrol Date: 2013-06-23 Impact factor: 3.714