Opinion statement
Purpose of the Review To provide an overview of recent advances in the treatment of pediatric antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Recent Findings With advances in research, use of standardized clinical assessment tools, advent of biologic therapies, and use of patient registries, concepts relating to the optimal management of pediatric patients with chronic primary systemic vasculitis have evolved and newer treatments strategies and treatment guidelines continue to emerge.
Conclusion Although most of what we have learned about pediatric AAV has come from adult data, the quality and breadth of pediatric data is accumulating because of multicenter, international collaborations. These efforts are critical given that optimal treatment strategies likely differ in the context of a developing immune system, and in a physically and emotionally developing child. Pragmatic clinical trials linked with international patient registries could help close the pediatric AAV evidence gap.