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ANCA‐small vessel vasculitides: what have we (not yet) learned from animal models?
Author(s) -
VAN DER VEEN BETTY S.,
HEERINGA PETER
Publication year - 2009
Publication title -
apmis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.909
H-Index - 88
eISSN - 1600-0463
pISSN - 0903-4641
DOI - 10.1111/j.1600-0463.2009.02472.x
Subject(s) - proteinase 3 , autoantibody , pathogenesis , in vivo , immunology , myeloperoxidase , animal model , medicine , in vitro , pathology , biology , inflammation , antibody , biochemistry , microbiology and biotechnology
Anti‐neutrophil cytoplasmic autoantibodies (ANCA) with a specificity for myeloperoxidase or proteinase 3 are closely associated with small vessel vasculitides (SVV). In vitro , ANCA activate primed neutrophils to release toxic substances that destroy endothelial cells, suggesting a pathogenic role for these autoantibodies in disease development. However, to study the complex interplay between ANCA, neutrophils, and the local environment in vivo , animal models are required. Here, we will review the animal models developed for ANCA‐SVV and discuss how these models have been applied to study ANCA‐SVV pathogenesis. In addition, some directions for future research pertaining to unresolved issues relevant for the pathogenesis and immunogenesis of ANCA‐SVV are proposed.