Agrawal Yuri, Godin David A, Belafsky Peter C
Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
J Voice. 2006 Sep;20(3):481-4. doi: 10.1016/j.jvoice.2005.10.010. Epub 2006 Feb 14.
Sarcoidosis is a multisystem disease with various clinical manifestations. It is characterized primarily on a histopathologic basis by the presence of noncaseating granulomata. Laryngeal involvement reportedly occurs in 3-5% of cases, and it is typically localized to the supraglottic region. Patients often present with hoarseness, dysphagia, stridor, or dyspnea. Laryngoscopy typically demonstrates a pale, edematous epiglottis studded with nodules. Tissue biopsy reveals the classic noncaseating granuloma; however, the finding is not diagnostic. Sarcoidosis remains a diagnosis of exclusion to be entertained once other verifiable etiologies for granulomatous disease of the larynx, such as TB, syphilis, fungal infection, berylliosis, or Wegener's granulomatosis, have been ruled out. Systemic corticosteroids remain the mainstay of treatment; however, new steroid-sparing therapies that target the inflammatory response of sarcoidosis are currently being investigated. The case history of a patient with laryngeal sarcoidosis who was managed with the immunosuppressant azathioprine (Imuran) is summarized along with a discussion of other treatment options.
结节病是一种具有多种临床表现的多系统疾病。其主要病理组织学特征为存在非干酪样肉芽肿。据报道,喉部受累在3%至5%的病例中出现,且通常局限于声门上区。患者常表现为声音嘶哑、吞咽困难、喘鸣或呼吸困难。喉镜检查通常显示会厌苍白、水肿,布满结节。组织活检可发现典型的非干酪样肉芽肿;然而,这一发现并无诊断特异性。一旦排除了喉肉芽肿性疾病的其他可证实病因,如结核病、梅毒、真菌感染、铍中毒或韦格纳肉芽肿病等,结节病仍需作为排除性诊断来考虑。全身用糖皮质激素仍然是主要的治疗方法;然而,目前正在研究针对结节病炎症反应的新型糖皮质激素节省疗法。本文总结了一名接受免疫抑制剂硫唑嘌呤(依木兰)治疗的喉结节病患者的病史,并讨论了其他治疗选择。