WS8_1 Otitis media with ANCA-associated vasculitis (OMAAV): A retrospective multicenter study in Japan, Clinical findings

Abstract

Objectives: ANCA-associated vasculitis (AAV) often involves initially middle ear and intractable otitis media is possible to be otitis media with AAV (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 disease. Methods: We performed a nationwide survey in Japan. Total 297 patients diagnosed with OMAAV from 65 departments, who initially visited between 1984 and 2014, were enrolled in this study. Results: 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. The follow-up period was ranged from 1 to 348 months with a median of 43.6 months. The most common ear symptoms at initial visit were hearing loss (98% patients). Other ear symptoms were tinnitus (50%), otorrhea (45%), otalgia (34%) and vertigo or dizziness (25%). These ear symptoms were resistant to treatments with antibiotics and tympanic tube. Other symptoms except for ear, facial palsy (18%), and hypertrophic pachymeningitis (15%) were observed. Nose (32%), lung (27%), kidney (18%) were also involved. During a clinical course, 43% patients experienced disease relapse. During the clinical course, facial palsy and hypertrophic pachymeningitis, which are characteristic symptoms in OMAAV, were 32% and 25%, respectively. Serum ANCA data were available in 289 patients at the initial visit. 55% were positive for MPO-ANCA, 22% for PR3-ANCA, 4% for both ANCA positive, and 19% for both ANCA negative. Definitive histological diagnosis was performed in only 30% patients. Eight (3%) patients died; 4 patients were disease related death and other 4 patients were treatment related death. In disease related death cases, 3 cases had subarachnoid hemorrhage with ANCA negative and hypertrophic pachymeningitis. One of them died without treatment, because he was not given the diagnosis. Conclusions: OMAAV has unique clinical features much different from the other intractable otitis media. 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.