The Role of Proteinase 3 and Neutrophils in ANCA-Associated Systemic Vasculitis
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منابع مشابه
Differentiation of Cocaine-Induced Midline Destructive Lesions from ANCA-Associated Vasculitis
Introduction: Cocaine-induced midline destructive lesions (CIMDL) are complications of regular nasal cocaine inhalation. CIMDL can mimic systemic diseases with positive anti-neutrophil cytoplasmic antibodies (ANCA), such as granulomatosis with polyangiitis (GPA). Case Report: In this article, we describe the case of a young woman who presented with nasal perforation induced by cocaine, al...
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Antineutrophil cytoplasm autoantibody (ANCA)-associated small vessel vasculitides are systemic diseases characterized by chronic inflammation of blood vessels. These vasculitides are associated with the presence of ANCA which are, in most cases, directed towards proteinase 3 (PR3) or myeloperoxidase (MPO). In vitro and in vivo data have suggested a pathophysiological role in the ANCA-associated...
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Antineutrophil cytoplasmic antibodies (ANCA) are predominantly IgG autoantibodies directed against constituents of primary granules of neutrophils and monocytes lysosomes. Although several antigenic targets have been identified, those ANCA directed to proteinase 3 or myeloperoxidase are clinically relevant, whereas the importance of other ANCA remains unknown. Both are strongly associated with ...
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Background: Wegener granulomatosis or granulomatosis with polyangiitis is a pauci-immune small vessel vasculitis which is usually associated with anti-neutrophil cytoplasmic antibodies (ANCA) mainly in old men. This small vessel vasculitis is usually characterized by necrotizing granulomatous inflammation with multiorgan involvement. Kidneys could be involved as the main and life-threatening co...
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Wegener's granulomatosis (WG) is an inflammatory disorder characterized by granulomatous inflammation and vasculitis, and is strongly associated with antineutrophil cytoplasmic antibodies (ANCA). ANCA in patients with WG are directed against proteinase 3 (Pr3) in most of the cases. In vitro, upon neutrophil priming, ANCA antigens are expressed on the cell surface, thereby becoming available for...
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