目的:肉芽肿性多血管炎与耳鼻咽喉科主诉的比例为70-95%,最常见的是浆液性中耳炎。在极少数情况下,患者可能会出现面神经麻痹并伴有耳科或鼻部症状;并且,经常,最初出现在耳鼻喉科医生。对于医疗保健专业人员来说,重要的是能够认识到面神经麻痹是肉芽肿性血管炎的潜在表现。
方法:系统评价。
方法:遵循系统审查和荟萃分析(PRISMA)方案的首选报告项目,PubMed和MED-LINE数据库查询了2007年1月至2022年12月发表的描述肉芽肿合并多血管炎的面神经麻痹的文章。以前被称为韦格纳肉芽肿病。关键词包括“面神经麻痹”,“面部麻痹”,“肉芽肿性多血管炎”,“韦格纳肉芽肿病”,标题/摘要中的“ANCA阳性”。所有英文全文文章都经过筛选,包括单个案例演示。摘要,评论,被认为超出我们研究目标范围的出版物被排除在审查范围之外。删除重复的文章后,共筛选了85篇文章。在应用纳入和排除标准后,14篇文章被纳入审查。
结果:文献中共有28例面神经麻痹的报道,这些患者最终被诊断为肉芽肿性多血管炎。患者年龄从14岁到68岁不等。没有一个病人以前被诊断为GPA,大多数患者最初出现其他耳科症状。据报道,24例患者(86%)出现听力损失。11例患者出现耳痛(39%),6例(21%)出现耳漏。文献中报道了10例患者(36%)的双侧面瘫。总的来说,16例面瘫患者接受手术治疗:6例鼓室吻合术,4例乳腺切除术,2探索性鼓室切开术。通常认为手术在解决面部无力方面无效。所有患者最终都接受了类固醇和免疫抑制剂的组合,最常见的是泼尼松龙和环磷酰胺或利妥昔单抗,最终过渡到硫唑嘌呤进行维持。与听觉阈值不同,在两名患者中仍然下降,所有患者在对血管炎进行适当的药物治疗后恢复了面部功能。
结论:肉芽肿性多血管炎患者的面神经麻痹是一种罕见但可治疗的现象。在顽固性中耳炎患者中,无法解决的面神经麻痹,或耳科问题的组合,重要的是要考虑GPA作为一个可能的来源。接受适当血管炎治疗的患者的面部功能预后良好,但需要进一步的研究来确认。
OBJECTIVE: Granulomatosis with polyangiitis is associated with otolaryngologic complaints in 70-95 % of cases, with the most common being serous otitis media. In rare cases, patients may experience facial nerve palsy in conjunction with otologic or nasal symptoms; and, often, initially present to an otolaryngologist. It is important for healthcare professionals to be able to recognize the nuisances of facial nerve palsy as a potential presentation of granulomatosis with polyangiitis.
METHODS: Systematic
review.
METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Protocol, PubMed and MED-LINE Databases were queried for articles published from January 2007 to December 2022 describing facial nerve palsy in the context of Granulomatosis with polyangiitis, formerly known as Wegener\'s Granulomatosis. The keywords included \"facial nerve palsy\", \"facial palsy\", \"granulomatosis with polyangiitis\", \"Wegener\'s granulomatosis\", \"ANCA positive\" in the title/abstract. All full-text articles available in English were screened, including single case presentations. Abstracts, commentaries, and publications deemed outside the scope of our study aims were excluded from
review. After removal of duplicate articles, a total of 85 articles were screened. After applying inclusion and exclusion criteria, 14 articles were included in the
review.
RESULTS: There were a total of 28 reports of facial nerve palsy in the literature in patients who were eventually diagnosed with granulomatosis with polyangiitis. The patients\' ages ranged from 14 to 68 years old. None of the patients had been previously diagnosed with GPA, and a majority of them presented initially with other otologic symptoms. Hearing loss was reported in 24 patients (86 %), otalgia was present in 11 patients (39 %), and otorrhea was present in 6 patients (21 %). Bilateral facial paralysis was reported in 10 patients in the literature (36 %). In total, 16 patients underwent surgery for facial paralysis: 6 tympanomastoidectomies, 4 mastoidectomies, 2 explorative tympanotomies. Surgery was generally considered ineffective in resolving facial weakness. All patients ended up receiving some combination of steroids and immunosuppressant, most commonly prednisolone and cyclophosphamide or rituximab, which was eventually transitioned to azathioprine for maintenance. Unlike auditory thresholds, which remained decreased in two patients, all patients recovered facial function following appropriate medical treatment of their vasculitis.
CONCLUSIONS: Facial nerve paralysis in patients with granulomatosis with polyangiitis is a rare but treatable phenomenon. In patients with intractable otitis media, unresolving facial palsy, or a combination of otologic issues, it is important to consider GPA as a possible source. The prognosis for facial function appears to be excellent in patients who undergo appropriate treatment for vasculitis, but further studies are needed for confirmation.