ANCA-associated vasculitis, pediatric

Abstract

Antineutrophil cytoplasmic autoantibody (ANCA)-driven vasculitis is associated with high morbidity in children and adolescents. Albeit rare, the disease warrants efficient diagnosis and aggressive treatment as more than half of these children experience irreversible organ damage and in particular chronic kidney disease. Distinct clinical phenotypes of this form of vasculitis exist, and the classification and definitions of these phenotypes have evolved over the decades. As new insights into the pathophysiology of ANCA-associated vasculitis grow, new potential therapies are slowly emerging.