Department of Otolaryngology-Head and Neck Surgery, Asahikawa Medical University, , Midorigaoka-Higashi 2-1-1-1, Asahikawa, 078-8510 Hokkaido, Japan.
Department of Otolaryngology-Head and Neck Surgery, Asahikawa Medical University, , Midorigaoka-Higashi 2-1-1-1, Asahikawa, 078-8510 Hokkaido, Japan.
Auris Nasus Larynx. 2021 Feb;48(1):2-14. doi: 10.1016/j.anl.2020.07.004. Epub 2020 Aug 5.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a form of necrotizing vasculitis with few or no immune deposits. It primarily affects small and medium blood vessels. AAV is classified into three categories, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangitis (EGPA), and two major ANCAs, proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA are involved in their pathogenesis. Intractable otitis media frequently occurs in patients with GPA, MPA or EGPA, although all patients show similar clinical features, regardless of the type of AAV. Furthermore, approximately 15% patients with otitis media caused by AAV do not show ANCA positivity, histopathological evidence, or any other AAV-related lesions at the initial visit; therefore, these patients do not fulfill the ordinary diagnostic criteria for systemic AAV. Thus, we first proposed that this condition could be categorized as "otitis media with AAV (OMAAV)". Subsequently, the Japanese Otological Society (JOS) conducted a nationwide survey between December 2013 and February 2014 and identified 297 patients with OMAAV. The survey revealed that OMAAV is a disease that initially occurs in the middle ear and subsequently spreads to other organs such as the lungs and kidneys, with eventual involvement of all body organs. Severe sequelae such as facial palsy, hypertrophic pachymeningitis, complete deafness, and subarachnoid hemorrhage resulting in death can also occur. In this review, we introduce the clinical features, diagnostic criteria, and treatment strategies recommended by JOS for early diagnosis and treatment of OMAAV.
抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）是一种坏死性血管炎，其免疫沉积物很少或没有。它主要影响小血管和中等血管。AAV 分为三种类型，肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA），两种主要的 ANCAs，蛋白酶 3（PR3）-ANCA 或髓过氧化物酶（MPO）-ANCA 参与其发病机制。难治性中耳炎常发生在 GPA、MPA 或 EGPA 患者中，尽管所有患者均表现出相似的临床特征，但无论 AAV 类型如何。此外，约 15%的由 AAV 引起的中耳炎患者在初次就诊时不表现出 ANCA 阳性、组织病理学证据或任何其他 AAV 相关病变；因此，这些患者不符合系统性 AAV 的一般诊断标准。因此，我们首先提出这种情况可以归类为“伴 AAV 的中耳炎（OMAAV）”。随后，日本耳科学会（JOS）于 2013 年 12 月至 2014 年 2 月期间进行了一项全国性调查，共确定了 297 例 OMAAV 患者。该调查显示，OMAAV 是一种最初发生在中耳的疾病，随后会扩散到肺部和肾脏等其他器官，最终累及所有器官。也可能会发生严重的后遗症，如面瘫、肥厚性硬脑膜炎、完全性耳聋和蛛网膜下腔出血导致死亡。在这篇综述中，我们介绍了 JOS 推荐的 OMAAV 的临床特征、诊断标准和治疗策略，以实现早期诊断和治疗。
