eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
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SCImago Journal & Country Rank
2/2020
vol. 45
 
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abstract:
Review paper

Antineutrophil cytoplasmic antibodies – their role in pathogenesis, diagnosis and treatment monitoring of ANCA-associated vasculitis

Justyna Fijołek
1
,
Elżbieta Wiatr
1

1.
The Third Department of Pneumonology, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland
Cent Eur J Immunol 2020; 45 (2): 218-227
Online publish date: 2020/07/09
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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by necrotizing inflammation of small blood vessels and the presence of ANCA. Increasing clinical and experimental evidences support their pathogenic role in AAV, but the exact mechanism is not fully understood. Recently, the important role of neutrophil extracellular traps (NETs) in pathogenesis of AAV is underlined. There is an indication that NETs can be a source for the formation of ANCA. The most common ANCA target antigens are myeloperoxidase (MPO) and proteinase 3 (PR3). Though the mechanism of action of ANCA is still under exploration, ANCA serology is being increasingly used for classification of AAV and revealed as kenner in defining various disease subsets associated with different genetic background, clinical features, treatment response, and prognosis. Controversy exists regarding the utility of serial measurements of ANCA in patients with AAV to monitor treatment and predict disease relapse.
keywords:

ANCA, vasculitis, pathogenesis, diagnosis, treatment monitoring

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