Recognition of ear, nose and throat involvement in patients with ANCA-associated vasculitis: from in-depth interviews to insights for the future.

PURPOSE: Ear, nose, and throat (ENT) manifestations are common in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Although timely intervention is crucial, diagnosis and treatment are often delayed due to its rarity and nonspecific symptoms. Currently, no standardized tool exists to assess AAV activity in the ENT region. This study aims to determine key indicators of active AAV in the ENT region, thereby supporting the development of a systematic examination method. METHODS: A qualitative exploratory multicentre study was conducted. In-depth interviews with four otorhinolaryngologists were thematically analysed. Identified ENT symptoms and rhinoscopic findings associated with AAV were compiled, and statements regarding diagnosis and management were formulated. These items then formed the basis of an online survey presented to Dutch otorhinolaryngologists. Respondents rated items on a 0-10 agreement scale. RESULTS: Of 29 initiated surveys, 25 (86.2%) were completed. Highest-rated symptoms supporting active AAV included nasal crusting (9.7/10), systemic symptoms (7.5/10), no effect of conventional therapy for chronic rhinosinusitis (7.6/10) and epistaxis (7.5/10). Most suspect rhinoscopic findings were nasal crusting (9.3/10), mucosal haemorrhaging (8.8/10) and septal perforation (8.6/10). Respondents strongly agreed that ENT involvement should be monitored by an otorhinolaryngologist (8.3/10), nasal lavage should precede rhinoscopy (7.6/10) and thorough nasal hygiene instructions should be provided (9.1/10). CONCLUSION: Key indicators of AAV activity include nasal crusting, lack of response to conventional treatment, systemic symptoms, nasal crusting, mucosal haemorrhaging and septal perforation. These data may facilitate the establishment of a standardized scoring system to optimize diagnostic accuracy and disease monitoring.