Pathogenesis and pathology of anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis
Daisuke Tsukui, Yoshitaka Kimura, Hajime Kono
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.
Topics & Concepts
PathogenesisVasculitisAnti-neutrophil cytoplasmic antibodyImmunologyAntibodyComplement systemPathogenicityMyeloperoxidaseCytoplasmInflammationMedicinePathologyBiologyMicrobiologyDiseaseBiochemistryVasculitis and related conditionsNeutrophil, Myeloperoxidase and Oxidative MechanismsInflammasome and immune disorders