ENT manifestations of Granulomatosis with polyangiitis

Ear, nose, and throat (ENT) symptoms represent the most frequent manifestations at the onset of granulomatosis with polyangiitis (GPA). The diagnosis of the localized form of GPA remains challenging thus contributing to the delay at the beginning of treatment. This could lead to major sequelae due to tissue destruction. Otolaryngologists play a key role in collaboration with rheumatologists in early GPA diagnosis and treatment. Clinicians should maintain a high index of suspicion and proceed to a more comprehensive evaluation in cases of imperfect response to treatment or with unexplained atypical findings. The challenging issue in localized organ involvement during GPA is to determine whether the non-specific local upper aerodigestive symptoms are caused by a specific or a non-specific inflammatory process. The role of surgery becomes crucial in cases of laryngeal involvement. Lifethreatening airway compromise necessitates urgent surgical intervention. There is an emerging role in the local ENT therapies.