Antineutrophil cytoplasmic autoantibodies, autoantigens, and systemic vasculitis.

摘要: Antineutrophil cytoplasmic antibodies (ANCA) encompass a heterogeneous group of autoantibodies targeting antigens in neutrophils (PMN), monocytes, and endothelial cells. ANCA are routinely detected by the indirect immunofluorescence technique (IFT) at least three different patterns fluorescence can be distinguished which have been assigned acronyms cANCA, pANCA aANCA. cANCA is mostly induced proteinase 3 (PR3) (PR3-ANCA), myeloperoxidase (MPO) (MPO-ANCA), while aANCA has unidentified subspecificity. Over past decade, subject extensive investigation. They proved to significant value both as diagnostic tools for follow-up several forms systemic vasculitis (e.g. Wegener's granulomatosis, WG; microscopic polyarteritis, MPA; Churg-Strauss syndrome, CSS) now termed 'ANCA-associated vasculitides'. Furthermore, it suspected that presence an important factor pathogenesis these disease groups. Data regarding detection their role vasculitic disorders will discussed this review. Growing evidence points pathophysiological relevance distribution target PR3 MPO (presence circulation, on cell membranes, tissue extracellularly). An autoimmune process implicated ANCA-associated vasculitis, but uncertain mechanism underlies induction ANCA-related immunoresponse. In paper mechanisms such antigenic cross-reactivity between human PMN proteins extrinsic molecular mimicry, idiotypic immunoglobulin regulation, T-cell reactivity discussed.