Orbital inflammatory diseases (OIDs) from various causes may have a similar clinical presentation, with orbital biopsy being a key investigation to assist with diagnosis. Granulomatosis with polyangiitis (GPA) is an idiopathic granulomatous inflammatory disease that forms part of the spectrum of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis. When left untreated, GPA is an aggressive disease, can result in severe, permanent organ damage, and is potentially life threatening when vital organs are affected.
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