What is granulomatosis with polyangiitis?
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Granulomatosis With Polyangiitis Previously called Wegener granulomatosis , granulomatosis with polyangiitis ( GPA ) is a necrotizing vasculitis charac-terized by a triad of the following: Necrotizing granulomas of the upper-respiratory tract (ear, nose, sinuses, throat) or the lower-respiratory tract (lung) or both Necrotizing or granulomatous vasculitis affecting small-to medium-sized vessels (e.g., capillaries, venules, arteri-oles, and arteries), most prominently the lungs and upper airways but other sites as well Focal necrotizing , often crescentic, glomerulonephritis . "Limited" forms of disease can be restricted to the respi ratory tract. Conversely, when widespread the disease may affect the eyes, skin, and other organs, most notably the heart; clinically, widespread GPA resembles PAN with the additional feature of respiratory involvement. GPA is likely initiated as a cell-mediated hypersensitivity response to inhaled infectious or envirorimental antigens. P...