RESUMO
BACKGROUND: Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) is a primary systemic vasculitis that affects medium to small sized vessels throughout the body. It often presents with symptoms and signs involving the ear, nose, and head and neck area. OBJECTIVE: To highlight salient features of ENT-related issues in granulomatosis with polyangiitis, and raise awareness of the condition. METHODS: A case report of a patient with limited disease and an insidious onset is presented, along with a review of the current literature. In addition, basic initial management is described. RESULTS: Eighty-six publications were used to describe salient features of ENT-related issues in granulomatosis with polyangiitis. CONCLUSION: The time to diagnosis has not reduced significantly in the last 10 years in the UK. A high index of suspicion is required for an earlier diagnosis of granulomatosis with polyangiitis.