Clinical characteristics of granulomatosis with polyangiitis with suppurative otitis media as the initial presentation in children

Purpose: To evaluate the specific clinical characteristics of granulomatosis with polyangiitis (GPA) especially those involving ear-nose-throat signs in children, suggest approaches for the treatment and emphasize the significance of clinical multidisciplinary management. Methods: : Retrospect the diagnosis and treatment process for a case of GPA with suppurative otitis media as the initial presentation; review the literature of this involvement regions; generalize an appropriate multidisciplinary diagnosis and treatment plan for such kind of disease in children. Results: : A 13-year-old child of GPA initially presented with suppurative otitis media and was prescribed anti-infection therapy for 2 months. Otologic symptoms were recurrent and other systematic presentations including skin rashes, eyelids swelling and pulmonary nodules gradually appeared. She was referred to nearly four different departments (pneumology, rheumatology, otorhinolaryngology and dermatology) and doctors found the level of PR3-ANCA increase remarkably. Undergoing evaluating comprehensively, the diagnosis of GPA was made finally. Conclusions: : GPA, a rare immune disease in children, is easily misdiagnosed and missed diagnosed for a long time resulting in delaying treatment. The general presentations and multi-systematic clinical characteristics of GPA are various. It is significant for the specific diagnosis of GPA that paying attention to multidisciplinary discussions and long-term follow up.