Highlights
AAV is characterized by necrotizing small vessel vasculitis with positive serum ANCA.
MPO/PR3-ANCA and neutrophils play central roles in AAV pathogenicity.
Dysregulated complement system primes neutrophils.
MPO-ANCA directly activates neutrophils to induce NETosis followed by releasing NETs.
B cells, T cells, and dendritic cells also contribute to the pathogenicity of AAV.