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Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Kimberly A Morishita1, Linda Wagner-Weiner2, Eric Y Yen3

  • 1British Columbia's Children's Hospital and the University of British Columbia, Vancouver, British Columbia, Canada.

Arthritis Care & Research
|March 6, 2021
PubMed

Insights

Standardized treatment plans for severe pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV) were established through consensus. These plans aim to facilitate future comparative effectiveness and safety studies in pediatric AAV management.

Area of Science:

  • Pediatric Rheumatology
  • Vasculitis Research
  • Clinical Consensus

Background:

  • Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) lacks standardized treatment protocols in pediatric populations.
  • The rarity of pediatric AAV precludes large-scale randomized controlled trials.
  • Severe pediatric AAV requires urgent and effective management strategies.

Purpose of the Study:

  • To establish consensus treatment plans (CTPs) for severe pediatric AAV.
  • To enable future studies on the comparative effectiveness and safety of AAV treatments.
  • To provide standardized approaches for managing new-onset granulomatosis with polyangiitis, microscopic polyangiitis, and renal-limited AAV in children.

Main Methods:

  • A dedicated AAV Workgroup of pediatric rheumatologists and nephrologists conducted a literature review.
  • Consensus conferences utilized nominal group techniques to define treatment strategies for remission induction and maintenance.
  • CARRA membership voted on the proposed CTPs, data collection, and outcome measures.

Main Results:

  • Two CTPs were developed for both remission induction and maintenance of severe pediatric AAV.
  • A glucocorticoid-weaning regimen, core data set, and outcome measures were defined.
  • CTPs for remission induction and maintenance achieved high approval rates (94% and 98%, respectively) from CARRA membership.

Conclusions:

  • Consensus methodology successfully established standardized CTPs for severe pediatric AAV.
  • The developed CTPs were broadly accepted by the CARRA membership.
  • These standardized plans will support pragmatic comparative effectiveness research in a long-term registry.
Abstract

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