Pathogenesis and pathology of anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis

Daisuke Tsukui; Yoshitaka Kimura; Hajime Kono

doi:10.1016/j.jtauto.2021.100094

Pathogenesis and pathology of anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis

J Transl Autoimmun. 2021 Mar 3:4:100094. doi: 10.1016/j.jtauto.2021.100094. eCollection 2021.

Authors

Daisuke Tsukui¹, Yoshitaka Kimura¹, Hajime Kono¹

Affiliation

¹ Department of Internal Medicine, Teikyo University School of Medicine, Tokyo, Japan.

Abstract

•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.

Keywords: ANCA-Associated vasculitis; Anti-neutrophil cytoplasmic antibody; NETs; Neutrophil.

Publication types

Review