Clinical features of OMAAV

-facial nerve palsy, hypertrophic pachymeningitis and relapse-

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by systemic necrotizing vasculitis. Recently, reports of otitis media with AAV (OMAAV) with bone-conduction hearing loss have increased and there is increasing recognition of OMAAV. Facial nerve palsy and hypertrophic pachymeningitis are symptoms that are frequently associated with OMAAV. We investigated the clinical features of OMAAV, particularly facial nerve palsy and hypertrophic pachymeningitis. Additionally, we examined the characteristics of relapse cases.
Of 297 cases, patients with facial nerve palsy numbered 94 (32%) and patients with hypertrophic pachymeningitis numbered 70 (24%). Many patients with facial nerve palsy had otorrhea, otalgia and headache as initial symptoms. The patients with hypertrophic pachymeningitis were also similar results. It was found that facial nerve palsy and hypertrophic pachymeningitis were relevant with otitis media due to vasculitis, in other words OMAAV. Concerning about multiple organ disorder, lung disorder was less in the patients with hypertrophic pachymeningitis and kidney disorder was less in the patients with facial nerve palsy. OMAAV with facial nerve palsy and/or hypertrophic pachymeningitis had different features from typical systemic GPA.
There were 125 cases (43%) with relapse in their clinical course. Relapsed cases had no characteristic symptom in this study. Although it was not examined in detail in this study, the relapsed organ was not necessarily coincided with the organ which impaired at the first time. There is a possibility that the various symptoms appear during the course. Therefore it is necessary to follow up taking care of the multiple organ symptoms after the initial treatment. Concerning treatment, patients treated with steroid therapy without immunosuppressive drugs relapsed more than patients treated with steroid and immunosuppressive drugs.
Facial nerve palsy and hypertrophic pachymeningitis were relatively common symptoms in OMAAV. These findings might be a key for the diagnosis. Furthermore, it was revealed that OMAAV can easily relapse. Initial immunosuppression therapy that includes corticosteroid is therefore essential for achieving long-term remission for OMAAV.