ANCA相关性血管炎伴中耳炎的临床特征与听力结局：一项包含未分类疾病的四组比较研究
Clinical Characteristics and Hearing Outcomes of Otitis Media With ANCA-Associated Vasculitis: A Four-Group Comparison Including Unclassified Disease.
文献信息
| PMID | 42847189 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Kento Komatsuda |
| 作者单位 | Department of Otolaryngology-Head and Neck Surgery, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan. |
| 期刊 | Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology |
| SCI 分区 | Q2 |
| IF | 1.9 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 耳科 |
中文摘要
目的： 描述抗中性粒细胞胞浆抗体相关性血管炎伴中耳炎（OMAAV）各疾病分类的临床特征、听力结局和复发模式，特别关注未分类OMAAV。
研究设计： 回顾性队列研究。
地点： 三级转诊大学医院。
患者： 2009年至2025年间诊断为OMAAV的56例患者（89耳），分类为嗜酸性肉芽肿性多血管炎（n=16）、肉芽肿性多血管炎（GPA，n=17）、显微镜下多血管炎（n=8）或未分类OMAAV（n=15）。
干预： 免疫抑制治疗，包括全身性皮质类固醇联合或不联合其他免疫抑制剂。
主要结局指标： 从疾病发作到开始治疗的时间、纯音测听结果以及耳部和全身复发的累积发生率。
结果： 与其他亚型相比，未分类OMAAV患者从发病到开始治疗的时间显著更长，就诊时听力损伤更严重。在整个队列中，较长的治疗开始时间与较重的基线听力损失呈中度相关。尽管就诊时听力损伤最严重，未分类OMAAV患者在免疫抑制治疗后听力改善与其他组相当。3年耳部复发累积发生率在各组间无显著差异。相比之下，全身性复发在GPA中最常见。在GPA患者中，时间分析显示耳部和全身复发模式异质，耳部复发并不总是先于全身性加重。
结论： 未分类OMAAV因孤立性耳部表现而容易诊断延迟，导致发病时严重听力损失；然而，对治疗的反应性保留凸显了早期诊断和及时免疫抑制治疗的重要性。在肉芽肿性多血管炎中，耳部复发并不总是先于全身性复发，强调无论耳部疾病活动如何，都需要仔细的全身评估。
英文摘要
OBJECTIVE: To characterize clinical features, hearing outcomes, and relapse patterns across disease categories of otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV), with particular focus on unclassified OMAAV.
STUDY DESIGN: Retrospective cohort study.
SETTING: Tertiary referral university hospital.
PATIENTS: Fifty-six patients (89 ears) diagnosed with OMAAV between 2009 and 2025, classified as eosinophilic granulomatosis with polyangiitis (n=16), granulomatosis with polyangiitis (GPA, n=17), microscopic polyangiitis (n=8), or unclassified OMAAV (n=15).
INTERVENTIONS: Immunosuppressive therapy, including systemic corticosteroids with or without additional immunosuppressants.
MAIN OUTCOME MEASURES: Time from disease onset to treatment initiation, pure-tone audiometric outcomes, and cumulative incidence of otologic and systemic relapse.
RESULTS: Patients with unclassified OMAAV demonstrated a significantly longer time to treatment initiation and more severe hearing impairment at presentation compared with those with other subtypes. Across the entire cohort, longer time to treatment initiation was moderately correlated with greater baseline hearing loss. Despite presenting with the most severe hearing impairment, patients with unclassified OMAAV showed hearing improvement comparable to that in the other groups following immunosuppressive therapy.The 3-year cumulative incidence of otologic relapse did not differ significantly among groups. In contrast, systemic relapse occurred most frequently in GPA. Among patients with GPA, temporal analysis revealed heterogeneous patterns of otologic and systemic relapse, and otologic relapse did not consistently precede systemic exacerbation.
CONCLUSIONS: Unclassified OMAAV is prone to diagnostic delay due to an isolated otologic presentation, resulting in severe hearing loss at onset; however, preserved responsiveness to treatment highlights the importance of early diagnosis and prompt immunosuppressive therapy. Otologic relapse does not consistently precede systemic relapse in granulomatosis with polyangiitis, underscoring the need for careful systemic evaluation regardless of otologic disease activity.