HLA genes in ANCA-associated vasculitides.

摘要: Primary systemic vasculitis affecting smaller vessels is usually associated with antineutrophil cytoplasmic antibodies (ANCA). The ANCA-associated vasculitides include Wegener's granulomatosis, microscopic polyangiitis, Churg Strauss syndrome and renal limited vasculitis. There considerable evidence that genetic factors influence susceptibility to vasculitis, including reports of familial cases, differences in racial incidence, associations polymorphic variants proteins such as alpha-1-antitrypsin. mounting evidence, from clinical vitro studies, ANCA may be pathogenic. However, it also clear autoreactive T cells are likely involved, by providing cell help for production possibly producing cell-mediated immune injury. Indeed, patients have been shown proliferate response the target antigens - proteinase 3 myeloperoxidase. In most T-cell-dependent autoimmune diseases there positive and/or negative HLA genes. These genes encoded major histocompatibility complex (MHC) their products, molecules, play a central role generation responses. For this reason, many investigators looked vasculitides. Problems analysing these definition concerned, varying methodology typing. A number reported striking no consistent association has identified different series. recent studies DR1, DQw7 or DR8, DR3 DR13, significant associations. This lack an obvious extremely interesting, suggests very heterogeneous, genuine strong yet identified. Further investigation problem clearly needed improve our understanding pathogenesis