Anti-neutrophil cytoplasmic autoantibodies and leukocyte-endothelial interactions: a sticky connection?
Anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificity for myeloperoxidase (MPO) or proteinase 3 (Pr3) are associated with systemic small-vessel vasculitides (SVV). Detection of ANCA is an established clinical tool in disease diagnosis and monitoring. Based on clinical and in vitro experimental evidence, a pathogenic role for ANCA has long been suspected, however, in vivo models in which causality can be tested have been lacking. Recently, an exciting novel rat model of MPO-ANCA-associated vasculitis has been described, which provides compelling evidence that MPO-ANCA are a primary pathogenic factor in SVV by augmenting leukocyte-endothelial interactions and vascular wall damage.
Heeringa
Peter
P
Cardiovascular Research Institute Maastricht, Division of Clinical and Experimental Immunology, Maastricht University, PO Box 616, 6200 MD Maastricht, The Netherlands. P.Heeringa@immuno.unimaas.nl
Huugen
Dennis
D
Tervaert
Jan Willem Cohen
JW
D000818:Animals; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D002448:Cell Adhesion / Q000276:immunology; D004195:Disease Models, Animal; D042783:Endothelial Cells / Q000276:immunology*; D006801:Humans; D007962:Leukocytes / Q000276:immunology*; D018448:Models, Immunological; D053146:Myeloblastin; D009195:Peroxidase / Q000276:immunology; D051381:Rats; D012697:Serine Endopeptidases / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase; D012697:Serine Endopeptidases; D053146:Myeloblastin