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: Biswanath Basu, T K S Mahapatra, Nirmal Mondal
• Journal: Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association (Nephrol Dial Transplant) Vol. 30 Suppl 1 Pg. i113-8 (Apr 2015) ISSN: 1460-2385 [Electronic] England
• PMID: 25758433 (Publication Type: Journal Article)
• Copyright: © The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Antibodies, Monoclonal, Murine-Derived
• Antineoplastic Agents
• Immunosuppressive Agents
• Rituximab
• Mycophenolic Acid

Topics

• Algorithms
• Antibodies, Antineutrophil Cytoplasmic (immunology)
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Antineoplastic Agents (therapeutic use)
• Child
• Child, Preschool
• Drug Therapy, Combination
• Female
• Follow-Up Studies
• Glomerular Filtration Rate
• Humans
• Immunosuppressive Agents (therapeutic use)
• Kidney Diseases (mortality, prevention & control)
• Male
• Microscopic Polyangiitis (drug therapy, mortality, pathology)
• Mycophenolic Acid (analogs & derivatives, therapeutic use)
• Remission Induction
• Retrospective Studies
• Rituximab
• Survival Rate
• Treatment Outcome