title: Predictors of relapse and long-term outcome in children with steroid-dependent nephrotic syndrome after rituximab treatment.
abstract: BACKGROUND
  In patients with complicated steroid-dependent nephrotic syndrome (SDNS), rituximab (RTX) followed by immunosuppressive agent (IS) can maintain remission without the use of prednisolone (PSL). However, available data on the predictive factors for relapse and the long-term outcome after this protocol are few.
  
  
  METHODS
  We retrospectively analyzed 43 SDNS patients who were followed-up for a long time (>2 years, mean 5.4 years) after a single dose of RTX (375 mg/m2) from September 2007. After RTX, PSL was tapered off within 6 months; monotherapy with IS, such as cyclosporine or mycophenolate mofetil, was continued to prevent post-RTX relapse. For patients who achieved >12 months of PSL-free remission, IS was also tapered off.
  
  
  RESULTS
  Thirty-nine patients (91 %) could discontinue PSL without relapses at a median of 154 days after the initial RTX. The first relapse of NS occurred in 39 patients (91 %) at a median of 586 days; additional RTX doses were administered in 28 patients (65 %). Kaplan-Meier analysis showed that shorter CD19 cell depletion (<150 days) and younger age at RTX initiation (<12.5 years) were significantly associated with high risk for first relapse after RTX (log rank p < 0.05). In multivariate analysis, mycophenolate mofetil therapy as maintenance IS after RTX was the only predicted risk factor for first relapse (hazard ratio 2.75; p = 0.027). At the last follow-up, IS was still used in 33 patients (77 %); treatment-free remission (>12 months) was achieved in only five patients (12 %).
  
  
  CONCLUSIONS
  The introduction of RTX may not be necessarily associated with improved long-term outcome.
authors: Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ku, Saitama city, Saitama, 339 8551, Japan. f_shuich@d2.dion.ne.jp.
authors: Shuichiro
authors: S
authors: Fujinaga
authors: Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan.
authors: Daishi
authors: D
authors: Hirano
authors: Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ku, Saitama city, Saitama, 339 8551, Japan.|Department of Pediatrics, Juntendo University School of Medicine, Tokyo, Japan.
authors: Akira
authors: A
authors: Mizutani
authors: Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ku, Saitama city, Saitama, 339 8551, Japan.
authors: Koji
authors: K
authors: Sakuraya
authors: Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ku, Saitama city, Saitama, 339 8551, Japan.
authors: Akifumi
authors: A
authors: Yamada
authors: Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ku, Saitama city, Saitama, 339 8551, Japan.
authors: Shunsuke
authors: S
authors: Sakurai
authors: Department of Pediatrics, Juntendo University School of Medicine, Tokyo, Japan.
authors: Toshiaki
authors: T
authors: Shimizu
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage; D018572:Disease-Free Survival; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007564:Japan; D053208:Kaplan-Meier Estimate; D008297:Male; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D011239:Prednisolone / Q000008:administration & dosage* / Q000009:adverse effects; D016016:Proportional Hazards Models; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab / Q000008:administration & dosage* / Q000009:adverse effects; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D016572:Cyclosporine; D011239:Prednisolone
keywords: Long-term outcome; Relapse; Rituximab; Steroid-dependent nephrotic syndrome