Localized Granulomatosis with Polyangiitis in A Young Adult: A Case Report

Background: Granulomatosis with polyangiitis (GPA) is an uncommon systemic vasculitis, rarely encountered in children and young adults, characterized by necrotizing granulomatous inflammation, classically involving a triad of airway, lung and renal disease. A localized form is now recognized as a distinct subtype with no renal or pulmonary involvement detected. Objective: To report a case of localized granulomatosis with polyangiitis in a young adult. Case: A 22-year-old male presented with a 12-year history of nasal discharge and congestion, nosebleed and headache, together with the sudden appearance of extensive black necrotic crusting within his nasal cavity and nose surface. Otorhinolaryngologic examination revealed septal perforation, saddle-nose deformity and palatal perforation. Chest Xray demonstrated no abnormality, but Computed Tomography Scan (CT-Scan) of the paranasal sinuses showed pansinusitis and his tissue biopsy revealed GPA. Management: Intravenous cyclophosphamide combined with high-dose methylprednisolone and other supportive treatments were administered. However, at his own parents' request, he was discharged from our hospital due to financial issue before fully completing treatments. Currently, the patient has died due to massive bleeding streaming down his nose and oropharynx, inflicting shortness of breath