Diagnostic process for otitis media with antineutrophil cytoplasmic antibody-associated vasculitis

Abstract

Otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is an autoimmune middle ear disease, which manifests as a subtype of ANCA-associated vasculitis (AAV). Approximately 60%, 20%, and 20% of the cases were MPO-ANCA-positive, PR3-ANCA-positive, and ANCA-negative, respectively. This article provides an overview of the current diagnostic and therapeutic approaches for OMAAV. The diagnostic criteria for AAV include the EMEA algorithm, Ministry of Health, Labour and Welfare diagnostic criteria, and 2022 ACR/EULAR classification criteria. However, the classification of MPO-ANCA-positive OMAAV cases remains challenging. Particularly, diagnosing cases that are ANCA-negative or limited to ear lesions without pulmonary or renal involvement is challenging, and applying the OMAAV diagnostic criteria is effective. For ANCA-negative cases, careful exclusion of differential diagnoses is essential, and diagnostic treatment with glucocorticoids (GC) may occasionally be considered based on the clinical response. According to standard AAV treatment protocols, remission induction with GC combined with rituximab or cyclophosphamide is recommended. In 2022, the introduction of avacopan, a selective C5a receptor inhibitor, offers benefits in reducing GC use and its associated side effects. Developing effective management strategies for OMAAV is crucial considering these diagnostic processes and treatment strategies.