[Wegener's granulomatosis and ANCA-associated vasculitis: pathogenic role of autoantigens, autoantibodies, and cytokines].

A real explosion of data is seen concerning the detection of autoantibodies collectively known as antineutrophil cytoplasmic antibodies (ANCA) recognizing granule proteins of granulocytes and monocytes. Distinct fluorescence staining patterns result from their diverse substrate specificities, and can even identify several disease entities mainly in the etiopathogenetically unclear group of primary systemic vasculitides (PSV). The well-documented disease specificity of ANCA recognizing proteinase 3 (PR3), and recent in vitro findings concerning the effect of cytokines on immunocompetent cells including the endothelium are the rationale for the presentation of a new model explaining the development of systemic vasculitis.