The DOI matches its Crossref record; 233 of the 233 references it cites were found in a registry or an index.
ANCA-associated vasculitis
Open accessAbstract
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of disorders involving severe, systemic, small-vessel vasculitis and are characterized by the development of autoantibodies to the neutrophil proteins leukocyte proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). The three AAV subgroups, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis and eosinophilic GPA (EGPA), are defined according to clinical features. However, genetic and other clinical findings suggest that these clinical syndromes may be better classified as PR3-positive AAV (PR3-AAV), MPO-positive AAV (MPO-AAV) and, for EGPA, by the presence or absence of ANCA (ANCA + or ANCA – , respectively). Although any tissue can be involved in AAV, the upper and lower respiratory tract and kidneys are most commonly and severely affected. AAVs have a complex and unique pathogenesis, with evidence for a loss of tolerance to neutrophil proteins, which leads to ANCA-mediated neutrophil activation, recruitment and injury, with effector T cells also involved. Without therapy, prognosis is poor but treatments, typically immunosuppressants, have improved survival, albeit with considerable morbidity from glucocorticoids and other immunosuppressive medications. Current challenges include improving the measures of disease activity and risk of relapse, uncertainty about optimal therapy duration and a need for targeted therapies with fewer adverse effects. Meeting these challenges requires a more detailed knowledge of the fundamental biology of AAV as well as cooperative international research and clinical trials with meaningful input from patients. The anti-neutrophil cytoplasmic antibody-associated vasculitides (AAVs) are autoimmune disorders characterized by inflammation and destruction of small blood vessels. In this Primer, the authors discuss the classification of AAVs and the pathogenetic mechanisms, diagnosis and treatment of these debilitating conditions.
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Cites
2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides
Jennette, J. C., Falk, R. J., Bacon, P. A., Basu, N., Cid, M. C., Ferrario, F., Flores‐Suarez, L. F., Gross, W. L., Guillevin, L., Hagen, E. C., et al. (2013) · Arthritis & Rheumatism · doi:10.1002/art.37715 · Cited by 6,631
Self-citationWegener Granulomatosis: An Analysis of 158 Patients
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Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis
Stone, J. H., Merkel, P. A., Spiera, R., Seo, P., Langford, C. A., Hoffman, G. S., Kallenberg, C. G. M., St. Clair, E. W., Turkiewicz, A., Tchao, N. K., et al. (2010) · New England Journal of Medicine · doi:10.1056/nejmoa0909905 · Cited by 2,762
Self-citationGenomic atlas of the human plasma proteome
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Rituximab versus Cyclophosphamide in ANCA-Associated Renal Vasculitis
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Self-citationNetting neutrophils in autoimmune small-vessel vasculitis
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Dynamic landscape of immune cell-specific gene regulation in immune-mediated diseases
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Chu, T.-Y., Zheng-Gérard, C., Huang, K.-Y., Chang, Y.-C., Chen, Y.-W., I, K.-Y., Lo, Y.-L., Chiang, N.-Y., Chen, H.-Y., Stacey, M., et al. (2022) · Nature Communications · doi:10.1038/s41467-022-34083-1 · Cited by 37
Huang, S. U.-S., & O’Sullivan, K. M. (2022) · doi:10.20944/preprints202203.0021.v1 · Cited by 65
Wang, L., Novoa-Laurentiev, J., Cook, C., Srivatsan, S., Hua, Y., Yang, J., Miloslavsky, E., Choi, H. K., Zhou, L., & Wallace, Z. S. (2025) · International Journal of Medical Informatics · doi:10.1016/j.ijmedinf.2025.105797 · Cited by 5
Exploration of the genetic architecture of ANCA-associated vasculitis
Byun, J., Shaw, V. R., Zhu, C., Han, Y., Sun, R., Zhang, Z., Xiao, X., Pinder, B. D., Zhang, J., Tadesse, Y., et al. (2026) · eBioMedicine · doi:10.1016/j.ebiom.2026.106502
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Metadata
- Authors
- Kitching, A. R., Anders, H.-J., Basu, N., Brouwer, E., Gordon, J., Jayne, D. R., Kullman, J., Lyons, P. A., Merkel, P. A., Savage, C. O. S., Specks, U., & Kain, R.
- Venue
- Nature Reviews Disease Primers
- Published
- 2020-08-27
- Volume
- 6
- Issue
- 1
- Pages
- 71-71
- ISSN
- 2056-676X (ISSN-L)
- OpenAlex
- W137802152
- Cited by
- 928
- References
- 233
- FWCI
- 22.22
- Type
- journal article
- Language
- en
- Open access
- green
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- Kitching, A. R., Anders, H.-J., Basu, N., Brouwer, E., Gordon, J., Jayne, D. R., Kullman, J., Lyons, P. A., Merkel, P. A., Savage, C. O. S., Specks, U., & Kain, R. (2020). ANCA-associated vasculitis. Nature Reviews Disease Primers. https://doi.org/10.1038/s41572-020-0204-y
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