Favourable renal survival in paediatric microscopic polyangiitis: efficacy of a novel treatment algorithm

Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association
Biswanath BasuNirmal Mondal

Abstract

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. 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. 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 pa...Continue Reading

References

Mar 24, 1999·Arthritis and Rheumatism·L GuillevinB Jarrousse
Aug 3, 2004·Pediatrics·UNKNOWN National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents
Oct 28, 2005·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Amira Peco-AnticBrankica Spasojevic
Mar 31, 2007·Kidney International·A Akcan-ArikanS L Goldstein
Jan 23, 2009·Journal of the American Society of Nephrology : JASN·George J SchwartzSusan L Furth
Jun 19, 2009·Rheumatology·Despina EleftheriouPaul A Brogan
Mar 20, 2010·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Anne-Laure Sellier-LeclercGeorges Deschênes
Jun 21, 2011·Pediatric Rheumatology Online Journal·Nishkantha ArulkumaranAlan D Salama
Jul 27, 2012·Pediatrics International : Official Journal of the Japan Pediatric Society·Kazumi YamatoTomoyuki Kawamura
Jul 6, 2014·The Journal of Rheumatology·Liangzhong SunTing Liu

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Citations

May 28, 2021·Clinical Journal of the American Society of Nephrology : CJASN·Marta CalatroniAugusto Vaglio

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