title: Effective treatment with rituximab for the maintenance of remission in frequently relapsing minimal change disease.
abstract: OBJECTIVE
  Treatment of frequently relapsing or steroid-dependent minimal change disease (MCD) in children and adults remains challenging. Glucocorticoids and/or other immunosuppressive agents are the mainstay of treatment, but patients often experience toxicity from prolonged exposure and may either become treatment dependent and/or resistant. Increasing evidence suggests that rituximab (RTX) can be a useful alternative to standard immunosuppression and allow withdrawal of maintenance immunosuppressants; however, data on optimal treatment regimens, long-term efficacy and safety are still limited.
  
  
  METHODS
  We undertook a prospective study of RTX to allow immunosuppression minimization in 15 young adults with frequently relapsing or steroid-dependent, biopsy-proven MCD. All patients were in remission at the start of treatment and on a calcineurin inhibitor. Two doses of RTX (1 gr) were given 6 months apart. A subset of patients also received an additional dose 12 months later, in order to examine the benefit of re-treatment. Biochemical and clinical parameters were monitored over an extended follow-up period of up to 43 months.
  
  
  RESULTS
  Median steroid-free survival after RTX was 25 months (range 4-34). Mean relapse frequency decreased from 2.60 ± 0.28 to 0.4 ± 0.19 (P < 0.001) after RTX. Seven relapses occurred, five of which (71%) when CD19 counts were greater than 100 µ. Immunoglobulin levels remained unchanged, and no major side effects were observed throughout the follow-up period.
  
  
  CONCLUSIONS
  Rituximab therapy is effective at maintaining prolonged steroid-free remission and reducing relapse frequency in this group of patients. Our study lends further support for the role of RTX in the treatment of patients with frequently relapsing or steroid-dependent MCD.
authors: University College London Centre for Nephrology, Royal Free Campus, University College London, London, UK. e.papakrivopoulou@ucl.ac.uk.|University College London Institute of Child Health, University College London, London, UK. e.papakrivopoulou@ucl.ac.uk.
authors: Eugenia
authors: http://orcid.org/0000-0001-9457-3202
authors: E
authors: Papakrivopoulou
authors: University College London Centre for Nephrology, Royal Free Campus, University College London, London, UK.|Nephrology Unit, Internal Medicine Department, Faculty of Medicine, Zagazig University, Zagazig, Egypt.
authors: Ali M
authors: AM
authors: Shendi
authors: University College London Centre for Nephrology, Royal Free Campus, University College London, London, UK.
authors: Alan D
authors: AD
authors: Salama
authors: University College London Centre for Nephrology, Royal Free Campus, University College London, London, UK.
authors: Maryam
authors: M
authors: Khosravi
authors: University College London Centre for Nephrology, Royal Free Campus, University College London, London, UK.
authors: John O
authors: JO
authors: Connolly
authors: University College London Centre for Nephrology, Royal Free Campus, University College London, London, UK.
authors: Richard
authors: R
authors: Trompeter
mesh_terms: D000293:Adolescent; D000328:Adult; D065095:Calcineurin Inhibitors / Q000008:administration & dosage / Q000009:adverse effects; D004334:Drug Administration Schedule; D016903:Drug Monitoring / Q000379:methods; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D010043:Outcome and Process Assessment, Health Care; D011446:Prospective Studies; D012074:Remission Induction / Q000379:methods; D000069283:Rituximab / Q000008:administration & dosage / Q000009:adverse effects; D055502:Secondary Prevention / Q000379:methods / Q000706:statistics & numerical data; D006113:United Kingdom
chemical_list: D065095:Calcineurin Inhibitors; D007166:Immunosuppressive Agents; D000069283:Rituximab
keywords: calcineurin inhibitor; glucocorticoid; minimal change disease; nephrotic syndrome; rituximab