OBJECTIVE: To investigate differences in immune activity based on the presence of multiple organ involvement in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and whether hearing outcomes are different between patients with AAV localized to the ear and patients with systemic AAV. STUDY DESIGN: Retrospective case review. SETTING: University hospital. PATIENTS: Twenty patients with otitis media with AAV (OMAAV) who met the criteria proposed by the OMAAV study group in Japan. MAIN OUTCOME MEASURE(S): Serum levels of C-reactive protein, ANCA titer, soluble interleukin-2 receptor levels, and hearing outcome. RESULTS: Thirteen patients had disease involvement of organs other than the ear (systemic OMAAV group); involvement was localized to the ear in seven patients (localized OMAAV group). Serum levels of C-reactive protein, ANCA titer, and soluble interleukin-2 receptor were not significantly different between the groups. Hearing levels at diagnosis and in remission were significantly worse in the localized OMAAV group compared with the systemic OMAAV group. Hearing gain was not significantly different between groups. CONCLUSION: It is suggested that immune activity in patients with AAV localized to the ear is equivalent to activity in patients with systemic AAV. Therefore, we may need treatment for OMAAV equal in intensity to that for systemic AAV. As the hearing level at diagnosis was worse in patients with AAV localized to the ear than in patients with systemic AAV, earlier diagnosis may be needed to improve hearing outcome.
OBJECTIVE: To investigate differences in immune activity based on the presence of multiple organ involvement in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and whether hearing outcomes are different between patients with AAV localized to the ear and patients with systemic AAV. STUDY DESIGN: Retrospective case review. SETTING: University hospital. PATIENTS: Twenty patients with otitis media with AAV (OMAAV) who met the criteria proposed by the OMAAV study group in Japan. MAIN OUTCOME MEASURE(S): Serum levels of C-reactive protein, ANCA titer, soluble interleukin-2 receptor levels, and hearing outcome. RESULTS: Thirteen patients had disease involvement of organs other than the ear (systemic OMAAV group); involvement was localized to the ear in seven patients (localized OMAAV group). Serum levels of C-reactive protein, ANCA titer, and soluble interleukin-2 receptor were not significantly different between the groups. Hearing levels at diagnosis and in remission were significantly worse in the localized OMAAV group compared with the systemic OMAAV group. Hearing gain was not significantly different between groups. CONCLUSION: It is suggested that immune activity in patients with AAV localized to the ear is equivalent to activity in patients with systemic AAV. Therefore, we may need treatment for OMAAV equal in intensity to that for systemic AAV. As the hearing level at diagnosis was worse in patients with AAV localized to the ear than in patients with systemic AAV, earlier diagnosis may be needed to improve hearing outcome.