Pathogenesis-based new perspectives of management of ANCA-associated vasculitis

While the pathogenesis of anti-neutrophil cytoplasmic autoantibody associated vasculitis (AAV) is still not fully elucidated, there is a mounting evidence that the process is initiated by inflammation and activation of innate immunity in the presence of predisposing factors, innate immunity abnormalities, aberrant responses of the adaptive immune system, and complement system activation. Biologics targeting inflammation-related molecules in the immune system have been explored to treat AVV, and these treatments have provided revolutionary advances. When focusing on the pathogenic mechanisms of AVV, this review presents the new findings regarding novel therapeutic approaches for the management of these conditions.