抗中性粒细胞胞质抗体相关血管炎性中耳炎

张玉忠; 赵欢娣; 陈耔辰; 任鹏宇; 侯薇; 张勤; 吴琼; 金玉莲; 杨军; 张青

doi:10.13201/j.issn.2096-7993.2022.08.013

抗中性粒细胞胞质抗体相关血管炎性中耳炎

- 1.
  西安交通大学第二附属医院耳鼻咽喉头颈外科(西安，710004)
- 2.
  陕西中医药大学附属医院耳鼻咽喉头颈外科
- 3.
  上海交通大学医学院附属新华医院耳鼻咽喉-头颈外科上海交通大学医学院耳科学研究所上海耳鼻疾病转化医学重点实验室
基金项目:
国家自然科学基金资助项目(No：81970891；82171137)；陕西省国际合作重点研发计划项目(No：2020KWZ-019)

详细信息

通讯作者: 张青，E-mail：zhangqing03@xinhuamed.com.cn

中图分类号: R764.21

收稿日期: 2022-03-04

刊出日期: 2022-08-03

Otitis media with antineutrophil cytoplasmic antibody-associated vasculitis

- 1.
  Department of Otorhinolaryngology Head and Neck Surgery, the Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, 710004, China
- 2.
  Department of Otorhinolaryngology Head and Neck Surgery, the Affiliated Hospital of Shaanxi University Chinese Medicine
- 3.
  Department of Otorhinolaryngology-Head and Neck Surgery, Xinhua Hospital, Shanghai Jiaotong University School of Medicine; Shanghai Jiaotong University School of Medicine Ear Institute; Shanghai Key Laboratory of Translational Medicine on Ear and Nose diseases

More Information

Corresponding author: ZHANG Qing, E-mail: zhangqing03@xinhuamed.com.cn

Received Date: 04 March 2022

Publish Date: 03 August 2022

摘要

摘要: 目的探讨抗中性粒细胞胞质抗体(ANCA)相关血管炎性中耳炎(OMAAV)的临床特征、诊断和治疗。方法报告3例OMAAV患者的诊治过程，结合文献探讨该疾病的临床特点和诊疗方案。结果本组3例患者发病年龄56~72岁，男性2例，女性1例。3例患者与普通中耳炎的不同之处在于：①2例鼓膜表面出现肉芽样外观和血性分泌物；②骨导听力进行性下降；③2例出现周围面神经麻痹；④实验室检查ANCA阳性；⑤对普通中耳炎的常规治疗无效，使用糖皮质激素联合免疫抑制剂治疗效果良好。结论 OMAAV临床较为少见，在临床诊治中容易发生误诊和漏诊；早期明确诊断并使用糖皮质激素联合免疫抑制剂是治疗的关键。有创检查和手术治疗可能会加重症状，不宜实施。
- 中耳炎 /
- 抗中性粒细胞胞质抗体 /
- 原发性血管炎
Abstract: Objective To analyze clinical features, diagnosis and treatment of otitis media with antineutrophil cytoplasmic antibody(ANCA) -associated vasculitis(OMAAV).Methods This study reported three OMAAV patients and discussed the diagnosis and treatment of OMAAV combined with the recent literature.Results Two males and one female were included. The age of these three patients ranged from 56 to 72 years. Their characteristics were as follows: ①tympanic membrane with granulation and bloody secretions in two patients; ②progressive bone-conducted hearing loss within a short period of time; ③facial palsy in two patients; ④the laboratory test of ANCA is positive; ⑤conventional treatment of otitis media is ineffective, while glucocorticoid combined with immunosuppressive therapy is effective.Conclusion OMAAV is a rare disease and prone to misdiagnosis. Early diagnosis and treatment with corticosteroids and immunosuppressants are critical. Invasive tests and surgery during the active phase of OMAAV may aggravate symptoms and should be avoided.
- otitis media /
- antineutrophil cytoplasmic antibody /
- primary vasculitis

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图 1 例1患者检查结果

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图 2 例2患者检查结果

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图 3 例3患者辅助检查结果图

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表 1 3例OMAAV患者的临床特征

项目	例1	例2	例3
性别	男	女	男
年龄/岁	56	56	72
首发症状	耳痛，耳漏，听力下降	右耳鸣，听力下降	眩晕
进展症状	双耳听力下降，面瘫	双耳耳鸣、听力下降	双耳耳痛、听力下降，面瘫
误诊	中耳炎、耳道肿物	分泌性中耳炎	分泌性中耳炎、感音神经性聋
常规中耳炎治疗	无效	无效	无效
ANCA	PR3-ANCA(+)	PR3-ANCA(+)	PR3-ANCA(+)
病理	(耳)外耳道炎性坏死组织	无	(肺)坏死性肉芽肿性炎
修正诊断	AAV；GPA；OMAAV；混合性听力下降	AAV；GPA；OMAAV；混合性听力下降	AAV；GPA；OMAAV；混合性听力下降
累及其他器官	肺	无	肺
治疗方案	糖皮质激素+环磷酰胺	糖皮质激素+环磷酰胺	糖皮质激素+环磷酰胺
预后	听力好转，面瘫未完全恢复	耳鸣减弱，听力恢复良好，高频听力未恢复	听力好转，面瘫未完全恢复

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