带有多胞膜炎的粒状瘤,呈现为急性双边面部神经
Nicholas Toomey1, Aaron J Done1, Doron Sagiv1
1Department of Otolaryngology-Head and Neck Surgery, University of California, Davis, USA.
The Annals of otology, rhinology, and laryngology
|September 27, 2025
概括
双边面部是一种罕见的Granulomatosis with polyangiitis (GPA) 的症状. 这一案例突出了GPA诊断,即使没有系统参与,强调中耳活检的准确诊断.
科学领域:
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
- 类风湿病学 类风湿病学
- 神经学 神经学
背景情况:
- 带有多管炎 (GPA) 的粒状瘤炎是一种罕见的自身免疫性血管炎,影响小血管.
- 面部是一种不常见的头部和部GPA的表现.
- 双边面部是一种极其罕见的GPA表现.
研究的目的:
- 报告一种罕见的双边面部病例作为GPA的初始表现.
- 提高人们对这种不寻常的GPA表现的认识.
主要方法:
- 一位患有双边面部的患者的回顾性图表审查.
- 诊断工作包括成像 (CT,MRI),实验室测试 (PR3-ANCA) 和中耳活检.
- 手术干预涉及面部神经减压的乳腺切除术.
主要成果:
- 一名52岁的女性出现了连续的双边面部,听力损失和耳.
- 图像检测显示了没有胆固醇瘤的不透明的乳头.
- 通过阳性PR3-ANCA和显示血管炎的中耳活检证实了GPA的诊断.
- 患者在接受Rituximab和Prednisone治疗后,面部神经功能显著改善.
结论:
- 这一病例代表了首次报告的由于GPA而导致双边面部的情况,而没有其他系统性参与.
- 缺乏全身症状和负面的初始自身免疫检测不应该排除GPA.
- 在这种情况下,重新评估系统性参与和受影响组织的活检对于诊断GPA至关重要.
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