以耳部症状首发、合并鼻咽占位的肉芽肿性多血管炎1例并文献复习

Granulomatosis with polyangiitis with initial otological symptoms combined with a nasopharynx lesion: a case report with literature review

目的 探讨1例临床少见的以耳部症状首发、合并鼻咽部占位的肉芽肿性多血管炎(GPA)的临床特征、实验室检查、病理表现及诊疗过程。方法 回顾性分析患者的病例资料,总结病例特点并回顾国内外GPA相关文献。结果 患者以中耳炎、迷路炎症状为首发表现,合并鼻咽部占位,病程中逐渐出现面瘫,三叉神经刺激症状加重。多次留取耳及鼻咽部活检示急慢性炎症细胞浸润。升级抗生素,同时为避免中耳炎侵犯岩骨及颅内行乳突开放术。中耳局部炎症改善后其耳痛、面瘫等仍不缓解,但激素治疗有效,遂进一步完善自免病相关检查并再次行鼻咽部活检,最终确诊为GPA,予激素及免疫抑制剂治疗得以控制症状。术后3个月暂无显著肺部及肾脏受累表现。结论 临床上发现不典型的中耳炎或常规治疗反复不愈,且逐渐进展出现内耳、颅神经侵犯表现如眩晕发作、面神经麻痹等,同时激素治疗有效,且合并鼻咽部占位、鼻窦炎影像学表现,或累及其他器官如肺、肾脏时,均应考虑到GPA的可能。当反复留取病理活检未能取得特异性确诊依据时,动态监测抗中性粒细胞胞浆抗体、红细胞沉降率、尿潜血、胸部CT、血肌酐等也具有重要的提示意义。

Objective To explore the clinical characteristics, laboratory examination, pathological features, diagnosis, and treatment of a rare case of granulomatosis with polyangiitis(GPA) with initial otological symptoms combined with a nasopharynx lesion. Methods This article retrospectively analyzed the clinical case, summarized its characteristics, and reviewed related literature at home and abroad. Results This patient?s earliest clinical symptoms included otitis media, labyrinthitis, and a nasopharynx lesion. Then came the facial paralysis, and the trigeminal nerve stimulation symptoms worsened gradually. Only acute and chronic infiltrating inflammatory cells were found in the middle ear and nasopharynx lesion biopsies. The antibiotics were then modified, and the mastoidectomy surgery was performed to avoid petrosal bone and cerebral invasion. Despite improvements in the local inflammation of the middle ear, the earache, facial palsy, and other symptoms did not improve. Glucocorticoid therapy, on the other hand, appeared to be helpful. As a result, we completed appropriate autoimmune disease examinations and had another nasopharynx biopsy. Finally, the GPA diagnosis was confirmed. Glucocorticoid and immunosuppressive treatments alleviated symptoms. For three months following surgery, there was no obvious evidence of lung or renal invasion. Conclusion In clinical practice, the diagnosis of GPA should be considered when certain atypical otitis media is repeatedly unhealed after conventional treatment but sensitive to glucocorticoids, along with nasopharynx lesions, sinusitis, lung or kidney involvement, and gradual invasion of the inner ear and cranial nerve manifesting as vertigo attacks and facial palsy. When repeated pathological biopsies fail to reveal specific diagnostic evidence, dynamic monitoring of anti-neutrophil cytoplasmic antibody, erythrocyte sedimentation rate, urine occult blood, chest CT, and serum creatinine are also important.