CAJ | 학술논문

目的：探讨以呼吸道损害为唯一表现的复发性多软骨炎( RP)的临床特征、诊断及治疗。方法分析我院1例经计算机体层摄影术CT及支气管软骨活检术后病理诊断的RP患者的临床资料，并结合文献进行复习。结果患者为老年男性，以发热、咳嗽、咳痰为主要表现，肺功能示阻塞性通气功能障碍，胸部CT示气管主支气管管壁增厚，纤维支气管镜检查示气管支气管软骨环消失、管腔狭窄，病理检查为慢性炎症。予以糖皮质激素治疗有效。结论 RP以呼吸道单独受累的较为罕见，而呼吸系统受累常提示预后不佳，是该疾病死亡的主要原因。呼吸系统首先受累时，诊断是困难的且病情容易被耽误。影像学检查及软骨活检有助于诊断。治疗方式包括糖皮质激素或糖皮质激素联合免疫抑制剂等药物治疗及纤维支气管镜下气道成形术。
목적：탐토이호흡도손해위유일표현적복발성다연골염( RP)적림상특정、진단급치료。방법분석아원1례경계산궤체층섭영술CT급지기관연골활검술후병리진단적RP환자적림상자료，병결합문헌진행복습。결과환자위노년남성，이발열、해수、해담위주요표현，폐공능시조새성통기공능장애，흉부CT시기관주지기관관벽증후，섬유지기관경검사시기관지기관연골배소실、관강협착，병리검사위만성염증。여이당피질격소치료유효。결론 RP이호흡도단독수루적교위한견，이호흡계통수루상제시예후불가，시해질병사망적주요원인。호흡계통수선수루시，진단시곤난적차병정용역피탐오。영상학검사급연골활검유조우진단。치료방식포괄당피질격소혹당피질격소연합면역억제제등약물치료급섬유지기관경하기도성형술。
Objective To discuss the clinical manifestations, diagnosis and treatment of patients with relap-sing polychondritis ( RP) only airway damage involved. Methods One case of RP confirmed by computerized tomo-graphy( CT) and cartilaginous biopsy in our hospital was reported. Relevant references about RP published were re-viewed. Results The patient was an old male, with clinical manifestations of fever, cough with sputum production. The pulmonary function test showed obstructive ventilatory disorder. Thoracic CT scan demonstrated a diffusely thick-ened tracheobronchial wall with tracheobronchial stenosis. Bronchoscopy revealed disappearance of tracheobronchial circle with airway stenosis and partly obliteration of both the trachea and main bronchi on expiration. Pathological re-sults were chronic inflammation. He was relieved by medication of corticosteroids. Conclusion Airway involvement without involving other organs of RP is rare, as the predictor of poor prognosis and high mortality. However, when the initial manifestation of RP is the airways, in most cases there will be a delay in the diagnosis. Image examinations and cartilage biopsy are very useful for diagnosis. Pharmacotherapy includes corticosteroids and immunodepressant. Endobronchial stenting can help to avoid severe airway obstruction in RP.