title: Favourable renal survival in paediatric microscopic polyangiitis: efficacy of a novel treatment algorithm.
abstract: BACKGROUND
  Microscopic polyangiitis (MPA) is one of the most common forms of antineutrophil cytoplasm autoantibodies (ANCA)-associated vasculitis in children. Cyclophospamide and glucocorticoid-based treatment protocols are still considered gold standard in managing this multi-system disorder. But treatment-related toxicity is a major cause of chronic morbidity and early mortality in MPA. Hence, the search for an effective and safe alternative immunosuppressant is essential.
  
  
  METHODS
  A retrospective analysis of baseline clinico-pathological presentation and treatment-outcome was performed among 11 paediatric MPA patients. All of whom were treated with a pre-specified cyclophosphamide free, rituximab- and mycophenolate mofetil (MMF)-based management protocol as per centre practice.
  
  
  RESULTS
  We describe the clinical course of 11 children with MPA over a median follow-up period of 20.9 months. Both patient survival and renal survival at 1 year follow-up were 100%. In spite of the varying degree of renal involvement at presentation, kidney function was recovered in all patients with a median estimated glomerular filtration rate (eGFR) of 79.5 mL/min/1.73 m(2). At last follow-up, 91% (10/11) of patients were in complete remission and one (9%) child continued partial remission state. There was no treatment failure. In total, 73% (8/11) of patients were off steroids at last follow-up and 82% (9/11) of patients never relapsed during follow-up period.
  
  
  CONCLUSIONS
  Efficacy and medium-term safety of rituximab- and MMF-based protocol in managing children with MPA was evident in this study.
authors.affiliation: Division of Pediatric Nephrology, Department of Pediatrics, NRS Medical College & Hospital, Kolkata, West Bengal 700014, India.
authors.forename: Biswanath
authors.initials: B
authors.lastname: Basu
authors.affiliation: Department of Pediatrics, NRS Medical College & Hospital, Kolkata, West Bengal 700014, India.
authors.forename: T K S
authors.initials: TK
authors.lastname: Mahapatra
authors.affiliation: Department of Community Medicine & Statistics, NRS Medical College & Hospital, Kolkata, West Bengal 700014, India.
authors.forename: Nirmal
authors.initials: N
authors.lastname: Mondal
mesh_terms: D000465:Algorithms; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007674:Kidney Diseases / Q000401:mortality / Q000517:prevention & control*; D008297:Male; D055953:Microscopic Polyangiitis / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D000069283:Rituximab; D009173:Mycophenolic Acid
keywords: Microscopic polyangiitis; paediatrics; rituximab