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

Ulf Schönermarck; Elena Csernok; Wolfgang L. Gross

doi:10.1093/ndt/gfu398

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

Ulf Schönermarck^*, Elena Csernok, Wolfgang L. Gross

^*Corresponding author for this work

Clinic of Rheumatology

Ludwig-Maximilians-Universität

33 Citations (Scopus)

Abstract

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.

Original language	English
Journal	Nephrology Dialysis Transplantation
Volume	30
Pages (from-to)	i46-i52
Number of pages	7
ISSN	0931-0509
DOIs	https://doi.org/10.1093/ndt/gfu398
Publication status	Published - 30.09.2015

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Academic Focus: Center for Infection and Inflammation Research (ZIEL)

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abstract = "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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Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis: Challenges and solutions 2014

Abstract

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