Ricerc@Sapienza

Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides of medium and small arteries, characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract with coexist- ing glomerulonephritis. We report a case of GPA in a patient presenting with a six-month history of spontaneous epistaxis, nasal obstruction and frontal headache. Nasal endoscopy showed a large nasal septum perforation and an anterior translucid mass in the right nasal fossa. Findings were confirmed by computed tomography (CT) scan with contrast. The patient underwent func- tional transnasal endoscopic removal of the mass; histological examination showed tissue features suggestive of GPA; dosage of c-ANCA e p-ANCA antibodies confirmed GPA diagnosis. Nasal septum perforation has long been recognized as a feature of GPA, in which granulomatous destruction of nasal cartilage can result in perforation and saddle-nose deformity. Prompt diagno- sis of GPA is important to initiate therapy which may be life-saving and organ sparing.