1. Nat. Rev. Nephrol. doi:10.1038/nrneph.2017.37
Figure 3 Proposed model for the interaction of anti-neutrophil cytoplasmic antibody
(ANCA), neutrophils and complement activation in the pathogenesis of ANCA-associated vasculitis
Figure 3 | Proposed model for the interaction of anti-neutrophil cytoplasmic antibody (ANCA), neutrophils and complement activation in the pathogenesis of ANCA-associated vasculitis. Priming of neutrophils by cytokines, such as C5a or tumour necrosis factor, leads to the translocation of ANCA antigens such as myeloperoxidase (MPO) or proteinase-3 (PR3) from the cytoplasm to the cell surface. ANCAs can further activate primed neutrophils to undergo respiratory burst and degranulation, and to release tissue factor (TF)-bearing microparticles and neutrophil extracellular traps (NETs). Neutrophil activation can lead endothelial cell injury, activation of the coagulation system, and activation of the alternative complement pathway via their cell membranes, microparticles and NETs. Activation of the alternative complement pathway and NETs in turn leads to the generation of C5a, which amplifies the inflammatory response through enhanced neutrophil recruitment and priming of neutrophils for ANCA-mediated activation.
Chen, M. et al. (2017) Complement in ANCA-associated vasculitis: mechanisms and implications for management
Nat. Rev. Nephrol. doi: /nrneph