Otorhinolaryngological manifestations of granulomatosis with polyangiitis in children

<b>Introduction:</b> Granulomatosis with polyangiitis (GPA) belongs to the group of necrotizing inflammations of small and medium-sized vessels associated with anti-neutrophil cytoplasmic antibodies (ANCAs). The disease is most prevalent in adults while symptoms originating from organs within the head and neck region are characteristic of the pediatric form.<b>Aim:</b> To analyze the available literature on granulomatosis with polyangiitis in the pediatric population, focusing primarily on symptoms originating from organs within the head and neck region, and to present the current criteria for the diagnosis of GPA.<b>Material and methods:</b> A review of literature on granulomatosis with polyangiitis in the pediatric population as published in years 1976–2023 and available via the Pubmed platform.<b>Results:</b> Granulomatosis with polyangiitis is rare within in the pediatric population. Symptoms originating from the upper respiratory tract and head and neck region, such as impaired nasal patency and bleeding, perforation of the nasal septum and subglottic stenosis of the larynx, are characteristic of the pediatric form. Upper and lower airway endoscopy is an important element in diagnostic management. Pediatric GPA is diagnosed according to the EULAR/PRINTO/PRES Ankara 2008 criteria.<b>Conclusions:</b> In the early stages of GPA, ENT symptoms are most prevalent in the pediatric patient populations, and therefore it is important that ENT specialists be familiar with the characteristic symptoms of the disease. GPA is a multisystem disease requiring multispecialty team cooperation in the diagnostic and treatment process.