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J Leukoc Biol. 2013 Oct;94(4):623-31. doi: 10.1189/jlb.1012525. Epub 2013 Feb 4.

The neutrophil in antineutrophil cytoplasmic autoantibody-associated vasculitis.

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  • 11.Virchow Klinikum Campus and Experimental and Clinical Research Center, Max-Delbrück Center for Molecular Medicine and the Charité Medical Faculty, Lindenberger Weg 80, 13125 Berlin, Germany.


Necrotizing, small-vessel vasculitis develops in patients with circulating ANCAs. Neutrophils and monocytes harbor the two major ANCA antigens, PR3 and MPO. A whole body of in vitro experiments implicated ANCA-activated neutrophil effector functions in the pathogenesis of vasculitis, whereas the role of monocytes is less well-characterized. Mouse models for anti-MPO-induced vasculitis were developed to study ANCA-neutrophil interactions in complex in vivo situations. We not only discuss the significance of ANCA-neutrophil interactions for disease induction but also how a detailed understanding of these interactions helps to identify novel treatment targets for ANCA vasculitis.


ANCA; animal model; crescentic glomerulonephritis; inflammation

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