title: Long-term follow-up after rituximab for steroid-dependent idiopathic nephrotic syndrome.
abstract: BACKGROUND
  In patients with refractory steroid-sensitive nephrotic syndrome (SSNS), treatment with rituximab has shown encouraging results; however, long-term follow-up data are not available.
  
  
  METHODS
  We performed a retrospective analysis of 37 patients (25 boys) with steroid-dependent nephrotic syndrome who were treated with rituximab (375 mg/m(2) given weekly for one to four courses). Long-term follow-up data (>2 years, median 36, range 24-92.8 months) are available for 29 patients (12 boys).
  
  
  RESULTS
  Twenty-six of 37 (70.3%) patients remained in remission after 12 months. Relapses occurred in 24 (64.8%) patients after a median of 9.6 (range 5.2-64.1) months. Time to first relapse was significantly shorter in patients receiving one or two compared to three or four initial infusions. In the 29 patients with long-term follow-up for >2 years, 12 (41%) patients remained in remission after the initial rituximab course for >24 months, 7 (24.1%) patients without further maintenance immunosuppression. Nineteen children received two to four repeated courses of rituximab increasing the total number of patients with long-term remission to 20 (69%), remission including 14 (48%) patients off immunosuppression. The proportion of patients with long-term remission was not related to the number of initial rituximab applications. No serious side effects were noted.
  
  
  CONCLUSIONS
  Rituximab is an effective treatment option in the short- and long-term control of treatment refractory SSNS. Further controlled studies are needed to address optimal patient selection, dose and safety of rituximab infusions.
authors.affiliation: Pediatric Nephrology, University Children’s Hospital, Hamburg, Germany. Kemper@uke.de
authors.forename: Markus J
authors.initials: MJ
authors.lastname: Kemper
authors.forename: Jutta
authors.initials: J
authors.lastname: Gellermann
authors.forename: Sandra
authors.initials: S
authors.lastname: Habbig
authors.forename: Rafael T
authors.initials: RT
authors.lastname: Krmar
authors.forename: Katalin
authors.initials: K
authors.lastname: Dittrich
authors.forename: Therese
authors.initials: T
authors.lastname: Jungraithmayr
authors.forename: Lars
authors.initials: L
authors.lastname: Pape
authors.forename: Ludwig
authors.initials: L
authors.lastname: Patzer
authors.forename: Heiko
authors.initials: H
authors.lastname: Billing
authors.forename: Lutz
authors.initials: L
authors.lastname: Weber
authors.forename: Martin
authors.initials: M
authors.lastname: Pohl
authors.forename: Katrin
authors.initials: K
authors.lastname: Rosenthal
authors.forename: Anne
authors.initials: A
authors.lastname: Rosahl
authors.forename: Dirk E
authors.initials: DE
authors.lastname: Mueller-Wiefel
authors.forename: Jörg
authors.initials: J
authors.lastname: Dötsch
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007223:Infant; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy*; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D013256:Steroids; D000069283:Rituximab