Pathogenesis of antineutrophil cytoplasmic antibodies-associated vasculitis

Łukasz Moos, Przemysław J. Kotyla



Antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) is a group of autoimmune disorders characterized by necrotizing small-vessel vasculitis, the presence of antineutrophil cytoplasmic antibodies (ANCA), and little or no staining for immunoglobulins and complements in vessel walls by immunofluorescence microscopy examination. Antineutrophil cytoplasmic antibodies are a group of autoantibodies, predominantly immunoglobulins G, against antigens in the cytoplasm of neutrophil granulocytes. The evidence supporting the pathogenicity of ANCA comes from clinical observations, in vitro studies and animal models. The origin of the ANCA autoimmune response is still under examination as there are many mechanisms responsible for the development of AAV which are still not fully understood. We are still accumulating new reports that allow us to better understand the pathogenetic pathways leading to the lesions found in AAV patients.

In this paper we reviewed the most probable mechanisms leading to the formation of ANCA, as well as the possible role of antibodies in the pathogenesis of vasculitides.


antineutrophil cytoplasmic antibodies; vasculitides

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