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喉气管受累作为复发性多软骨炎的初始表现。

Laryngotracheal involvement as the initial manifestation of relapsing polychondritis.

作者信息

Chang Shu-Jen, Lu Chong-Chen, Chung Yu-Mei, Lee Shinn-Shing, Chou Chung-Tei, Huang De-Feng

机构信息

Section of Allergy, Immunology, and Rheumatology, Department of Medicine, Cheng Hsin Rehabilitation Medical Center, Taipei Veterans General Hospital, Taipei, Taiwan, R.O.C.

出版信息

J Chin Med Assoc. 2005 Jun;68(6):279-82. doi: 10.1016/S1726-4901(09)70151-0.

Abstract

Relapsing polychondritis (RP) is a rare multisystemic disease characterized by recurrent inflammation of cartilaginous and noncartilaginous tissues. When laryngotracheal or bronchial cartilages are involved, the disease can be life-threatening and needs aggressive treatment. Upper airway complaints are rare as initial presentations of RP. Here, however, we present a case of RP, with initial manifestations of cough and dyspnea that were treated as bronchial asthma for 6 months. Subglottic stenosis was found in April 2003, during a bronchoscopic examination, and emergency tracheostomy was performed. Auricular and nasal chondritis and bilateral scleritis developed 3 months after tracheostomy. High doses of methylprednisolone and immunosuppressive agents were used, and active inflammation in the eyes and ears was controlled, but the patient's upper airway was completely collapsed. This case is reported with the hope of increasing awareness about the potential for early upper airway involvement in RP.

摘要

复发性多软骨炎(RP)是一种罕见的多系统疾病,其特征为软骨组织和非软骨组织反复发生炎症。当喉气管或支气管软骨受累时,该病可能危及生命,需要积极治疗。上气道症状作为RP的初始表现较为罕见。然而,在此我们报告一例RP病例,其初始表现为咳嗽和呼吸困难,被误诊为支气管哮喘并治疗了6个月。2003年4月在支气管镜检查时发现声门下狭窄,并进行了紧急气管切开术。气管切开术后3个月出现耳廓和鼻软骨炎以及双侧巩膜炎。使用了大剂量甲泼尼龙和免疫抑制剂,眼耳的活动性炎症得到控制,但患者的上气道完全塌陷。报告该病例旨在提高对RP早期上气道受累可能性的认识。

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