BACKGROUNDGranulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with typical respiratory tract and renal manifestations. We present the atypical case of a patient with pulmonary-renal syndrome associated with antiglomerular basement membrane (GBM) antibodies and proteinase 3 ANCA (PR3-ANCA).
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