RESUMO
INTRODUCTION: Granulomatosis with polyangiitis (GPA) is rare but debilitating autoimmune disease and commonly presents with sinonasal as well as other head and neck symptoms. AIMS: To summarize the ear, nose, and throat-specific symptomatology and management of GPA. METHODS AND RESULTS: We performed a literature review by using the PubMed search engine to provide a summary of recent and important literature that is pertinent to an otolaryngologist's clinical practice. We provide a guide on the pathophysiology, epidemiology, clinical features, investigation, and management (operative and nonoperative) of this important disease. CONCLUSIONS: This review illustrates the important role that an otolaryngologist can play in the work up and symptom management of patients with GPA. Knowledge of the common presenting symptoms as well as more rare presentations of GPA is extremely important for otolaryngologists as prompt diagnosis and management is extremely important to avoid significant morbidity and mortality.