Department of Otolaryngology, Head and Neck Surgery, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, Poland.
Graduate of Advanced MD Program, Poznan University of Medical Sciences, Poznan, Poland.
Eur Arch Otorhinolaryngol. 2022 Sep;279(9):4271-4278. doi: 10.1007/s00405-021-07166-w. Epub 2021 Nov 19.
The aim of this study was to present a series of 6 patients with facial nerve palsy and masked mastoiditis which constituted as revelators of localized granulomatosis with polyangiitis (GPA) and to evaluate the utility of the ACR/EULAR 2017 provisional classification criteria for GPA in such cases.
Study group included 58 patients with GPA. Cases with facial nerve palsy and masked mastoiditis were thoroughly analyzed.
The mean age of patients was 37 years. All manifested unilateral facial nerve palsy and hearing loss, while only 2 reported aural complaints suggesting inflammatory cause of the disease. All cases were qualified for surgical intervention. Intraoperative findings were similar: granulation tissue in tympanic cavity and/or pneumatic spaces of the mastoid process. Only 50% of histopathological results suggested vasculitis. In all cases, elevated levels of antineutrophil cytoplasmic antibodies (ANCA) against peroxidase 3 (PR3-ANCA) were determined. Two patients presented rapid progression of the disease and died within 1 week and 2 months, respectively. Four other patients manifested gradual improvement of hearing and facial nerve function after treatment.
GPA should be included into differential diagnosis in all cases of persistent facial nerve palsy especially when otological symptoms coexist. Even localized GPA could be very aggressive, revelating generalized form of the disease. Rapid systemic treatment of GPA can protect hearing and facial nerve from permanent severe dysfunction. The ACR/EULAR 2017 provisional classification criteria for GPA seem to be valuable tool in diagnosing ENT patients with localized otological form of the disease.
本研究旨在介绍 6 例面神经麻痹和隐蔽性乳突炎患者，这些患者是局限性肉芽肿性多血管炎（GPA）的表现，并评估 ACR/EULAR 2017 年 GPA 临时性分类标准在这类病例中的应用价值。
研究组包括 58 例 GPA 患者。对面神经麻痹和隐蔽性乳突炎的病例进行了详细分析。
患者的平均年龄为 37 岁。所有患者均表现为单侧面神经麻痹和听力损失，而仅有 2 例患者报告了耳部疾病提示疾病的炎症性病因。所有病例均符合手术干预的条件。术中发现相似：鼓室和/或乳突气房内有肉芽组织。仅 50%的组织病理学结果提示血管炎。在所有病例中，均检测到抗中性粒细胞胞质抗体（ANCA）针对过氧化物酶 3（PR3-ANCA）的升高水平。有 2 例患者疾病快速进展，分别在 1 周和 2 个月内死亡。另外 4 例患者在治疗后听力和面神经功能逐渐改善。
在所有持续性面神经麻痹的病例中，尤其是当存在耳科症状时，应将 GPA 纳入鉴别诊断。即使是局限性 GPA 也可能非常具有侵袭性，揭示出全身性疾病。快速的 GPA 全身治疗可以防止听力和面神经永久性严重功能障碍。ACR/EULAR 2017 年 GPA 临时性分类标准似乎是诊断具有局部耳科表现的 ENT 患者的有用工具。
