Therapeutic advances forantineutrophil cytoplasmic antibody-associated vasculitis in children

Antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV) is a group of systemic autoimmune diseases characterized by inflammation and destruction of small/medium-sized blood vessels with a presence of circulating ANCA.AAV comprises several clinical entities with diverse clinical presentations and yet therapeutic interventions are similar.Current individualized treatment of AAV is usually divided into two phases comprised of inducing and maintaining remission.Therapeutic options of pediatric AAV are based largely upon physician experiences and adult trials.This review summarized the pathogenesis, disease activity, injury assessment and recent therapeutic advances of pediatric AAV.