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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. PR3-ANCA...