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Biswanath Basu, T. K. S. Mahapatra, Nirmal Mondal, Favourable renal survival in paediatric microscopic polyangiitis: efficacy of a novel treatment algorithm, Nephrology Dialysis Transplantation, Volume 30, Issue suppl_1, April 2015, Pages i113–i118, https://doi.org/10.1093/ndt/gfv016
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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/1.73m2. 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.
Efficacy and medium-term safety of rituximab- and MMF-based protocol in managing children with MPA was evident in this study.
- mycophenolate mofetil
- immunosuppressive agents
- renal function
- vasculitis
- antineutrophil cytoplasmic autoantibody
- cyclophosphamide
- glucocorticoids
- autoantibodies
- child
- cytoplasm
- follow-up
- neoadjuvant therapy
- pediatrics
- steroids
- treatment failure
- kidney
- morbidity
- mortality
- microscopic polyarteritis nodosa
- rituximab
- toxic effect
- glomerular filtration rate, estimated
- complete remission
- partial response
- multisystem disorders
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