Department of Otolaryngology, Head and Neck Surgery, Ehime University Graduate School of Medicine, Toon, Japan; Department of Otolaryngology, Head and Neck Surgery, Ehime Prefectural Central Hospital, Matsuyama, Japan.
Department of Otolaryngology, Head and Neck Surgery, Ehime University Graduate School of Medicine, Toon, Japan.
Auris Nasus Larynx. 2021 Oct;48(5):885-889. doi: 10.1016/j.anl.2021.01.023. Epub 2021 Feb 13.
The concept of otitis media with ANCA-associated vasculitis (OMAAV) was recently proposed by the study group of the Japan Otological Society. However, little is known about the effect of ear involvement on the clinical features and prognosis of AAV. We investigate this issue in this study.
We retrospectively examined 36 patients diagnosed with OMAAV and 44 patients diagnosed with AAV without ear involvement (non-OMAAV) at Ehime University Hospital from 2013 to 2018. We collected serological findings including ANCA type and titer, C-reactive protein (CRP), serum creatinine level, organ involved at initial diagnosis, treatment, remission, disease relapse, and mortality from medical records. We investigated whether clinical features and outcomes differed between the OMAAV and non-OMAAV groups.
Age, ANCA titer, and CRP at initial diagnosis were not significantly different between the two groups, and the rate of intravenous cyclophosphamide (IVCY) use also did not differ. The proportions of patients with concurrent eye involvement, facial palsy (FP), and hypertrophic pachymeningitis (HCP) were significantly higher in the OMAAV than in the non-OMAAV group (p = 0.005, 0.005 and 0.049, respectively), while both renal and peripheral nerve involvement were significantly less common in OMAAV patients (p = 0.04). Among the 30 patients with renal involvement, serum creatinine level at diagnosis was significantly lower in the OMAAV group (p = 0.04). The mortality rate was 8.3% in OMAAV and 6.8% in non-OMAAV cases, but this difference was not significant. The rate of relapse was 33.3% in OMAAV and 13.6% in non-OMAAV cases; this difference was significant (p = 0.04).
Serological measurements of disease activity did not differ between the groups. Eye involvement, FP, and HCP, however, were significantly more common in AAV with ear involvement. In addition, renal involvement was less common and renal impairment was milder in AAV with ear involvement. These findings can be considered clinical features. The relapse rate was significantly higher in AAV with ear involvement.
日本耳科学会研究小组最近提出了伴抗中性粒细胞胞浆抗体（ANCA）相关性血管炎的中耳炎（OMAAV）这一概念。然而，关于耳部受累对血管炎的临床特征和预后的影响，人们知之甚少。我们在本研究中对此问题进行了调查。
我们回顾性地检查了 2013 年至 2018 年在爱媛大学医院诊断为 OMAAV 的 36 例患者和诊断为无耳部受累的 AAV（非 OMAAV）的 44 例患者。我们从病历中收集了血清学发现，包括抗中性粒细胞胞浆抗体类型和滴度、C 反应蛋白（CRP）、血清肌酐水平、初诊时受累的器官、治疗、缓解、疾病复发和死亡率。我们调查了 OMAAV 组和非 OMAAV 组之间的临床特征和结果是否存在差异。
两组间初诊时年龄、抗中性粒细胞胞浆抗体滴度和 CRP 无显著差异，静脉环磷酰胺（IVCY）使用率也无差异。OMAAV 组患者并发眼部受累、面瘫（FP）和肥厚性硬脑膜炎（HCP）的比例明显高于非 OMAAV 组（p=0.005、0.005 和 0.049），而肾脏和周围神经受累明显较少见（p=0.04）。在 30 例有肾脏受累的患者中，OMAAV 组患者的诊断时血清肌酐水平明显较低（p=0.04）。OMAAV 组死亡率为 8.3%，非 OMAAV 组死亡率为 6.8%，但差异无统计学意义。OMAAV 组的复发率为 33.3%，非 OMAAV 组的复发率为 13.6%；差异有统计学意义（p=0.04）。
两组疾病活动的血清学测量无差异。然而，眼部受累、FP 和 HCP 在伴耳部受累的血管炎中更为常见。此外，伴耳部受累的血管炎中肾脏受累较少，且肾损害较轻。这些发现可视为临床特征。伴耳部受累的血管炎的复发率明显较高。
