以呼吸道损害为唯一表现的复发性多软骨炎1例并文献回顾

Analysis of relapsing polychondritis with airway involvement alone

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。

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.