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 Antineutrophil cytoplasmic antibody-associated vasculitides and respiratory  disease Gomez-Puerta JA; Hernandez-Rodriguez J; Lopez-Soto A; Bosch XChest  2009[Oct]; 136 (4): 1101-1111Vasculitides associated with serum positivity for antineutrophil cytoplasmic  antibodies (ANCAs) are a well-established subgroup affecting small- to  medium-sized vessels that are commonly recognized as ANCA-associated vasculitis,  which includes necrotizing granulomatous vasculitis (NGV) [formerly Wegener  granulomatosis], microscopic polyangiitis (MPA), and Churg-Strauss syndrome. NGV  usually starts as a granulomatous disease of the respiratory tract and progresses  to systemic disease with proteinase 3 (PR3)-ANCA-associated vasculitis,  suggesting an aberrant cell-mediated immune response to exogenous or endogenous  antigens in the respiratory tract and resulting in granuloma formation. In NGV,  granulomata may represent lymphoid structures ultimately responsible for PR3-ANCA  production. In both NGV and MPA, necrotizing glomerulonephritis and necrotizing  pulmonary capillaritis may well result from an injury orchestrated by ANCA.  Untreated NGV and MPA normally are rapidly progressive and fatal. Pulmonary  capillaritis with alveolar hemorrhage is a severe complication in patients with  MPA and NGV. Because plasma exchange removes circulating ANCAs and other proteins  from the blood, its use has been advocated in critical situations of severe renal  and pulmonary involvement. However, no studies of plasma exchange in  ANCA-associated vasculitis focused on pulmonary involvement have been reported.  Dissecting the mechanisms of inflammation may identify molecular targets for  future therapies in ANCA-associated vasculitis. Thus, biological agents are  emerging as potential therapies in refractory cases. Notably, rituximab and  infliximab have been trialed with apparent initial clinical success.|Abatacept[MESH]|Antibodies, Antineutrophil Cytoplasmic/*immunology[MESH]|Antibodies, Monoclonal, Murine-Derived[MESH]|Antibodies, Monoclonal/therapeutic use[MESH]|Humans[MESH]|Immunoconjugates/therapeutic use[MESH]|Immunologic Factors/therapeutic use[MESH]|Lung Diseases/drug therapy/*etiology/mortality[MESH]|Prognosis[MESH]|Rituximab[MESH]|Tumor Necrosis Factor-alpha/antagonists & inhibitors[MESH]|Vasculitis/*immunology[MESH]
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