Journal
JOURNAL OF LEUKOCYTE BIOLOGY
Volume 108, Issue 2, Pages 617-626Publisher
OXFORD UNIV PRESS
DOI: 10.1002/JLB.3VMR0220-438RR
Keywords
azurophil granules; degranulation; myeloperoxidase; proteinase 3; vasculitis
Categories
Funding
- IFs foundation - Swedish Pharmacy Society
- Magnus Bergwall foundation [MS 2018-02579]
- Colliander foundation
- University of British Columbia (UBC) Four Year Fellowship (4YF)
- UBC-affiliated, Andrew Nord Fellowship in Rheumatology
- UBC Faculty of Medicine Summer student research program (SSRP) fellowship
- Centre for Blood Research (CBR) Summer student program fellowship
- Michael Smith Foundation for Health Research Scholar Award
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Neutrophils are the most abundant leukocytes in circulation and are key first responders in the immune response to infectious and non-infectious stimuli. Unlike other immune cells, neutrophils can mount a robust response (including a change in surface markers and the production of extracellular traps and reactive oxygen species) just minutes after sensing a disturbance. It has been speculated that, in some individuals, the activation of neutrophils inadvertently leads to the generation of anti-neutrophil cytoplasmic autoantibodies (ANCA) against particular neutrophil proteins (antigens) such as myeloperoxidase (MPO) and proteinase 3 (PR3). In these individuals, continuous ANCA-antigen interactions are thought to drive persistent activation of neutrophils, chronic immune activation, and disease, most notably, small vessel vasculitis. There are significant gaps however in our understanding of the underlying mechanisms and even the pathogenicity of ANCA given that vasculitis can develop in the absence of ANCA, and that ANCA have been found in circulation in other conditions with no apparent contribution to disease. These gaps are particularly evident in the context of human studies. Herein, we review knowledge on neutrophil-derived ANCA antigens PR3 and MPO, ANCA generation, and ANCA-antigen interaction(s) that may promote immune activation and disease.
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