Otolaryngological Features of ANCA Associated Vasculitis

Abstract

Objective: ANCA-associated vasculitis (AAV) is characterized by systemic necrotizing vasculitis. AAV is composed of proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA. Because infections are a trigger of AAV, the upper airway areas are the favorite sites of AAV. In this study, we compare the otolaryngological features of AAV between patients with PR3-ANCA and patients with MPO-ANCA. Method: Retrospective case series study. Thirty-four patients enrolled in this study. All patients were diagnosed with AAV at Hokkaido University between 2002 and 2009. We evaluated clinical otologic and rhinologic manifestation of AAV and compared the difference between 2 different ANCA positive patients. Results: The otologic symptoms were divided into 3 types: chronic otitis media (COM), secretory otitis media (SOM), and sensory nerve hearing loss (SNHL). The COM type was more frequently found in patients with PR3-ANCA ( P < .05). Conclusion: A large difference was observed in the disease spectrum between patients with PR3-ANCA and those with MPO-ANCA. In particular, the discrepancy in the granuloma formation between these 2 ANCAs was thought to be the main reason for these clinical feature differences in the otolaryngological field.