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Systemic Vasculitides: Current Status and Perspectives pp 141–158Cite as

ANCA-Associated Vasculitis and the Mechanisms of Tissue Injury

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Abstract

ANCA associated vasculitides (AAVs) comprise four different disease entities: I) granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis), II) microscopic polyangiitis (MPA), III) eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome), and IV) renal-limited vasculitis or isolated pauci-immune necrotizing and crescentic glomerulonephritis (NCGN). Experimental data support the notion that ANCA-induced activation of both neutrophils and monocytes is one of the main pathogenic mechanisms involved in disease induction. Binding of ANCA IgG to surface expressed ANCA antigens on myeloid cells leads to generation of reactive oxygen species (ROS), degranulation and activation of proteases, and formation of neutrophil extracellular traps (NET). Finally, activation of the complement system in AAVs by ANCA stimulated neutrophils leads to generation of C5a, which plays an important role in an amplifying inflammatory loop.

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  1. Experimental and Clinical Research Center (ECRC) at the MDC Berlin, Charité Berlin, Lindenberger Weg 80, 13125, Berlin, Germany

    Adrian Schreiber & Mira Choi

  2. Department of Nephrology and Intensive Care Medicine, Campus Virchow Clinic, Medical Faculty of the Charité, Berlin, Germany

    Adrian Schreiber & Mira Choi

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  1. Adrian Schreiber
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  2. Mira Choi
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Correspondence to Adrian Schreiber .

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Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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© 2016 Springer International Publishing Switzerland

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Schreiber, A., Choi, M. (2016). ANCA-Associated Vasculitis and the Mechanisms of Tissue Injury. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_13

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