title: Late recovery of renal function by rituximab in a patient with Wegener's granulomatosis.
abstract: BACKGROUND
  Rituximab has proven effective in the treatment of complicated granulomatosis with polyangiitis (Wegener's, GPA). Two controlled trials in adults demonstrated beneficial effects of rituximab compared to cyclophosphamide in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis to induce remission and to treat relapses. Pediatric experience with rituximab in GPA is limited; the impact on renal function is unknown.
  
  
  METHODS
  We report a female adolescent with GPA and necrotizing glomerulonephritis that lead to end-stage renal disease (ESRD). After 22 months of peritoneal dialysis, she still experienced relapses and major treatment-associated adverse effects. After a single dose of rituximab, she rapidly achieved clinical remission and, unexpectedly, steadily recovered glomerular filtration rate, plateauing at 25 ml/min/1.73 m(2). Peritoneal dialysis could be discontinued for 16 months.
  
  
  CONCLUSIONS
  This case documents a potent beneficial effect of rituximab on renal manifestation of GPA even in long-established ESRD.
authors.affiliation: Division of Pediatric Nephrology, Center for Pediatric and Adolescent Medicine, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany.
authors.forename: Michal
authors.initials: M
authors.lastname: Malina
authors.forename: Betti
authors.initials: B
authors.lastname: Schaefer
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Waldherr
authors.forename: Elke
authors.initials: E
authors.lastname: Wühl
authors.forename: Franz
authors.initials: F
authors.lastname: Schaefer
authors.forename: Claus Peter
authors.initials: CP
authors.lastname: Schmitt
mesh_terms: D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000187:drug effects* / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D010530:Peritoneal Dialysis; D020127:Recovery of Function; D000069283:Rituximab; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab