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Pathogenesis and therapeutic interventions for ANCA-associated vasculitis.
Title: | Pathogenesis and therapeutic interventions for ANCA-associated vasculitis. |
Authors: | Nakazawa, Daigo Browse this author | Masuda, Sakiko Browse this author | Tomaru, Utano Browse this author →KAKEN DB | Ishizu, Akihiro Browse this author →KAKEN DB |
Issue Date: | Feb-2019 |
Publisher: | Springer Nature |
Journal Title: | Nature reviews. Rheumatology |
Volume: | 15 |
Issue: | 2 |
Start Page: | 91 |
End Page: | 101 |
Publisher DOI: | 10.1038/s41584-018-0145-y |
PMID: | 30542206 |
Abstract: | Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) affects systemic small vessels and is accompanied by the presence of ANCAs in the serum. This disease entity includes microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and drug-induced AAV. Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors. The mechanism by which ANCAs cause vasculitis involves ANCA-mediated excessive activation of neutrophils that subsequently release inflammatory cytokines, reactive oxygen species and lytic enzymes. In addition, this excessive activation of neutrophils by ANCAs induces formation of neutrophil extracellular traps (NETs). Although NETs are essential elements in innate immunity, excessive NET formation is harmful to small vessels. Moreover, NETs are involved not only in ANCA-mediated vascular injury but also in the production of ANCAs themselves. Therefore, a vicious cycle of NET formation and ANCA production is considered to be involved in the pathogenesis of AAV. In addition to this role of NETs in AAV, some other important discoveries have been made in the past few years. Incorporating these new insights into our understanding of the pathogenesis of AAV is needed to fully understand and ultimately overcome this disease. |
Type: | article (author version) |
URI: | http://hdl.handle.net/2115/74654 |
Appears in Collections: | 保健科学院・保健科学研究院 (Graduate School of Health Sciences / Faculty of Health Sciences) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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Submitter: 石津 明洋
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