Ryu Changwan, Herzog Erica L, Pan Hongyi, Homer Robert, Gulati Mridu
Department of Internal Medicine, Section of Pulmonary, Critical Care, and Sleep Medicine, Yale University School of Medicine, New Haven, CT, USA.
Department of Pathology, Yale University School of Medicine, New Haven, CT, USA.
Am J Case Rep. 2017 Feb 13;18:157-159. doi: 10.12659/ajcr.902231.
BACKGROUND Laryngeal sarcoidosis is a rare extrapulmonary manifestation of sarcoidosis, accounting for 0.33-2.1% of cases. A life-threatening complication of laryngeal sarcoidosis is upper airway obstruction. In this report we describe our experience in the acute and chronic care of a patient who required an emergent tracheostomy, with the aim to provide further insight into this difficult to manage disease. CASE REPORT A 37-year-old African American female with a 10-year history of stage 1 sarcoidosis presented with severe dyspnea. Laryngeal sarcoidosis was diagnosed three years previously, and she remained stable on low-dose prednisone until six months prior to admission, at which time she self-discontinued her prednisone for the homeopathic treatment Nopalea cactus juice. Her physical examination was concerning for impending respiratory failure as she presented with inspiratory stridor and hoarseness. Laryngoscopy showed a retroflexed epiglottis obstructing the glottis with edematous arytenoids and aryepiglottic folds. Otolaryngology performed an emergent tracheostomy to secure her airway and obtained epiglottic biopsies, which were consistent with sarcoidosis. She was eventually discharged home on prednisone 60 mg daily. Following months of corticosteroids, a laryngoscopy showed the epiglottis continuing to obstruct the glottis. The addition of methotrexate to a tapered dosage of prednisone 10 mg daily was unsuccessful, and she remains on prednisone 20 mg daily for disease control. CONCLUSIONS Laryngeal sarcoidosis, a rare extrapulmonary manifestation of sarcoidosis, uncommonly presents as the life-threatening complication of complete upper airway obstruction. As such, laryngeal sarcoidosis is associated with significant morbidity and mortality, requiring a high index of suspicion for timely diagnosis and treatment.
背景 喉结节病是结节病罕见的肺外表现，占病例的0.33 - 2.1%。喉结节病的一种危及生命的并发症是上呼吸道梗阻。在本报告中，我们描述了对一名需要紧急气管切开术患者进行急性和慢性护理的经验，旨在为这种难以管理的疾病提供进一步的见解。病例报告 一名37岁非裔美国女性，有10年1期结节病病史，出现严重呼吸困难。三年前诊断为喉结节病，她一直服用低剂量泼尼松维持病情稳定，直到入院前六个月，此时她自行停用泼尼松，改用顺势疗法诺帕莱仙人掌汁治疗。她的体格检查提示即将发生呼吸衰竭，因为她出现吸气性喘鸣和声音嘶哑。喉镜检查显示会厌后倾阻塞声门，杓状软骨和杓会厌襞水肿。耳鼻喉科进行了紧急气管切开术以确保气道通畅，并获取了会厌活检组织，结果与结节病相符。她最终出院，每天服用60毫克泼尼松。经过数月的皮质类固醇治疗后，喉镜检查显示会厌仍阻塞声门。在逐渐减量至每日10毫克泼尼松的基础上加用甲氨蝶呤未取得成功，她目前仍每日服用20毫克泼尼松以控制病情。结论 喉结节病是结节病罕见的肺外表现，很少表现为危及生命的完全上呼吸道梗阻并发症。因此，喉结节病与显著的发病率和死亡率相关，需要高度怀疑以进行及时诊断和治疗。
