以上气道受累为首发表现的结节病四例并文献复习

目的 了解以上气道(鼻/鼻窦、咽、喉、中耳)受累为发表现的结节病的临床特点,以减少特殊表现结节病的误诊和漏诊.方法 回顾性分析北京同仁医院经组织病理学确诊的以上气道受累为首发表现的结节病4例,复习以“上气道结节病”为首发或主要表现的文献报道,分析上气道结节病的临床特征及诊断方法.结果 4例患者中女性3例,男性1例,年龄分别为52、53、34和15岁,分别以“鼻腔肿物”、“慢性中耳炎”、“声音嘶哑”和“慢性扁桃体炎”就诊;体格检查及影像学检查发现3例伴有肺、淋巴结等器官受累;经支气管黏膜活检、外周肿大淋巴结活检并行耳鼻咽喉病变部位的组织活检最终明确结节病诊断.以“结节病”为关键词,应用CNKI数据库检索中文文献(1915-2011),发现7篇结节病累及上气道的文献报道,均为结节病累及鼻.以相同关键词应用Pubmed数据库检索英文文献,发现结节病累及上气道的发生率为2.3％～6.0％,常伴有肺、淋巴结等其他器官病变,而以中耳、鼻、咽喉症状为首发表现者仅见个案报告.结论 以上气道受累为首发表现的结节病临床少见,是“常规治疗无效”的慢性耳鼻咽喉疾病的病因之一.详细的病史询问、体格检查和必要的辅助检查,特别是对该病特殊表现的充分认识,有助于减少误诊和漏诊.

Sarcoidosis with upper airway involvement as the presenting symptoms: case report and literature review

Objective To investigate the clinical characteristics of sarcoidosis with upper airway (including nose/paranasal sinus,pharynx,larynx and middle ear) involvement as the presenting symptoms,and therefore to minimize the misdiagnosis of sarcoidosis with special manifestations.Methods Four cases of sarcoidosis with upper airway involvement as the presenting symptoms diagnosed at our hospital were described.The clinical data were analyzed and related literatures were reviewed.Results Three female patients aged 52,53,34 years and one 15-year-old male patient,with the main complaints as “mycteric mass”,“chronic otitis media”,“hoarseness” and “chronic tonsillitis” respectively,were referred to our hospital for further evaluation.The diagnosis of sarcoidosis was finally confirmed by biopsy of bronchial mucous membrane, enlarged peripheral lymph nodes, and otorhinolaryngological lesions. Physical examination and imaging findings showed involvements of the lungs and/or the lymph nodes in 3 patients.Seven Chinese articles about upper airway sarcoidosis ( all involving the nose) were found after literature search with “sarcoidosis” as the key word at the CNKI database (1915 -2011 ).English literature search with the same key word at “Pubmed” showed that the rate of upper airway involvement in sarcoidosis varied from 2.3％ -6.0％,mostly concurrent with thoracic and lymph node diseases,whereas cases of sarcoidosis presenting with otorhinolaryngological symptoms were occasionally reported.Conclusions Although upper airway sarcoidosis was rare,it may be the cause of chronic otorhinolaryngological disease which responded poorly to routine treatments. Careful collection of medical history,physical examination and necessary accessory examinations,especially better understanding of the exceptional manifestations of sarcoidosis,can help to minimize misdiagnosis and therefore to improve the prognosis of the patients.