The Clinical Features of Otitis media with ANCA associated vasculitis (OMAAV)

Abstract

ANCA - associated vasculitis (AAV) often involves initially middle ear and intractable otitis media is possible to be OMAAV. However, clinical characteristics of OMAAV have not been clarified yet. In this study, we aimed to describe a series of OMAAV patients and underline the difficulties involved in diagnosing and treating this challenging disease. In this multicenter study, we assigned 297 patients (86 males and 211 females) with OMAAV from 65 departments of otolaryngology in Japan. Their age were ranged from 13 to 89 years. At the initial visit, ear, nose, other upper respiratory tract, lung, and kidney lesions were involved in 294 (97%), 95 (32%), 24 (6%), 80 (27%), 55 (18%) patients, respectively. Facial nerve palsy and hypertrophic pachymeningitis were observed in 54 (18%) and 44 (15%) patients at the initial visit, respectively. Serum PR3-ANCA and MPO-ANCA were detected in 76 (27%) and 169 (59%) patients, respectively. A definitive histological diagnosis of AAV could be done in only 54 (30%) patients. All patients responded to treatments with prednisolone and/or immunosuppression therapy. Three (1%) patients died from subarachnoid hemorrhage due to vasculitis of basiar artery. It is difficult to make definitive diagnosis of OMAVV. We found that facial nerve palsy, hypertrophic pachymeningitis and MPO-ANCA positivity are characteristic in OMAAV. We proposed the diagnostic criteria of OMAAV from these findings.