Otorhinolaryngological manifestations in granulomatosis with polyangiitis (Wegener's)

Granulomatosis with polyangiitis (Wegener's, GPA) is an uncommon disease of unknown etiology classically involves the ELK triad of the ear, nose, throat (E), lungs (L) and kidneys (K) with necrotizing granulomatous inflammation and vasculitis. Most of the initial symptoms begin in the head and neck region with a wide spectrum of involvement of any site ranging from the nasal septum, paranasal sinuses, oral mucosa, larynx and even the external, middle and internal ear. Diagnosis may be delayed because the onset is heterogeneous and sometimes limited to one organ. The pathologic findings of a characteristic inflammatory reaction pattern, and the serum findings of elevated antineutrophil cytoplasmic antibodies can help to establish the diagnosis. The differentiation from other conditions that mimic GPA such as lymphoma and infections is of critical importance to initiate appropriate treatment. Treatment of the underlying disease is medical with the use of immunosuppressive agents and will not be reviewed here. This review focuses on the otorhinolaryngologic manifestation and complication of GPA as well as their surgical management and specifies the role of the otorhinolaryngologist as an integral member of the multidisciplinary care team for patients with GPA.