Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis: challenges and solutions 2014

Anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificity for proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are a defining feature of ANCA-associated vasculitides (AAV). They play a pivotal role in disease pathophysiology and have strongly improved early diagnosis and treatment of t...

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Published inNephrology, dialysis, transplantation Vol. 30 Suppl 1; no. suppl_1; pp. i46 - i52
Main Authors Schönermarck, Ulf, Csernok, Elena, Gross, Wolfgang L
Format Journal Article
LanguageEnglish
Published England 01.04.2015
Subjects
Adeno-associated virus
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - etiology
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - immunology
Autoimmunity - immunology
Complement System Proteins - immunology
Humans
Neutrophils - immunology
pathogenesis
NETs
ANCA
vasculitis
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Summary:Anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificity for proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are a defining feature of ANCA-associated vasculitides (AAV). They play a pivotal role in disease pathophysiology and have strongly improved early diagnosis and treatment of these infrequent, but potentially fatal diseases. Neutrophils and their products are major players in initiating the autoimmune response and tissue destruction in vasculitic as well as granulomatous inflammation. This review highlights recent findings on old and novel players (ANCA, neutrophils, neutrophil extracellular traps, fibroblasts, immune cells and complement) and puts them into context with the current understanding of disease mechanisms in AAV.
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ISSN:0931-0509
1460-2385
DOI:10.1093/ndt/gfu398