Anti-neutrophil cytoplasmic antibodies and pathogenesis of small vessel vasculitides.
Anti-neutrophil cytoplasmic antibodies (ANCA) are a heterogenous group of autoantibodies with a broad spectrum of clinically associated diseases. ANCA testing has been established as a useful tool for the diagnosis of small vessel vasculitides, especially of 'ANCA-associated vasculitides' (AAV), such as Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome, in which circulating ANCA are commonly found. Within the last 20 years these antibodies were subject of intensive studies and a growing body of evidence arose for a distinct role of ANCA in the pathogenesis of the AAV. Our current concept of whether ANCA directly or indirectly contribute to vascular damage (ANCA-cytokine-sequence-theory) was mainly developed from in vitro studies and is supported by data from clinical investigations as well as animal models. Recently a direct causal link between ANCA and the development of glomerulonephritis and vasculitis has been demonstrated. We now know that a passive transfer of ANCA is sufficient to induce disease, but it remains to be discovered how the autoantibodies to neutrophil antigens might triggered.
Csernok
Elena
E
Department of Rheumatology, University of Lübeck, Oskar-Alexander-Str. 26, 24576 Bad Bramstedt, Germany. csernok@rheuma-zentrum.de
D000818:Animals; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D015267:Churg-Strauss Syndrome / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D006801:Humans; D053146:Myeloblastin; D009195:Peroxidase / Q000276:immunology; D012697:Serine Endopeptidases / Q000276:immunology; D014657:Vasculitis / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase; D012697:Serine Endopeptidases; D053146:Myeloblastin