title: Rituximab for minimal change disease in adults: long-term follow-up.
abstract: BACKGROUND
  Minimal change nephropathy or disease (MCD) accounts for 10-15% of cases of the nephrotic syndrome in adults with frequent relapses occurring in up to 25% of cases. The drug of choice is glucocorticoids (GCs), but GC-dependence is seen in 25-30%. Treatment with rituximab has been found to be effective in relapsing and GC-dependent cases, but little data are available regarding long-term outcome in adults.
  
  
  METHODS
  We present nine female and seven male patients, ranging from 19 to 73 years of age with multirelapsing, GC-dependent or GC-resistant disease with a kidney biopsy consistent with MCD. Twelve patients were steroid-dependent with a lowest daily GC dose between 5 and 20 mg/day.
  
  
  RESULTS
  Rituximab with a total dose 1000-2800 mg divided in two to four doses was given together with GC achieving B-cell depletion before the second dose. No major side-effects occurred. Thirteen of the patients responded with complete remission enabling discontinuation or tapering of GC significantly below levels, where relapses had occurred in the past (P < 0.001). Two patients reached partial remission and one had no response to therapy. Follow-up was 12-70 months (median 44). Eight patients have remained in remission, whereas relapses occurred in seven patients after 9-28 months with repeated rituximab treatment in four of these.
  
  
  CONCLUSIONS
  Our study reinforces the role of rituximab as a GC-sparing agent in the challenging GC-dependent and multirelapsing MCD patients. In this emerging therapeutic field randomized studies with extended follow-up will add important information regarding optimal treatment, relapse and safety.
authors.affiliation: Division of Renal Medicine, K56, Department of Clinical Science, Karolinska University Hospital at Huddinge, Interventions and Technology (CLINTEC), Karolinska Institutet, Stockholm, Sweden.
authors.forename: Annette
authors.initials: A
authors.lastname: Bruchfeld
authors.forename: Samiha
authors.initials: S
authors.lastname: Benedek
authors.forename: Marie
authors.initials: M
authors.lastname: Hilderman
authors.forename: Charlotte
authors.initials: C
authors.lastname: Medin
authors.forename: Sunna
authors.initials: S
authors.lastname: Snaedal-Jonsdottir
authors.forename: Maarit
authors.initials: M
authors.lastname: Korkeila
mesh_terms: D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D018951:Antigens, CD20; D001706:Biopsy; D002908:Chronic Disease; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D007275:Injections, Intravenous; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000188:drug therapy* / Q000473:pathology; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D005938:Glucocorticoids; D007155:Immunologic Factors; D000069283:Rituximab
keywords: B-cell depletion; glucocorticoid sparing; long-term outcome; minimal change disease; relapse; rituximab; safety