气道受累的复发性多软骨炎的临床分析  

Clinical analysis of relapsing polychondritis with airway involvement

作　　者：褚海青[1] 赵兰[1] 任胜祥[1] 刘锦铭[1] 易祥华[2] 

机构地区：[1]同济大学附属肺科医院呼吸内科,上海200433 [2]同济大学附属肺科医院病理科,上海200433

出　　处：《同济大学学报（医学版）》2008年第4期109-112,120,共5页Journal of Tongji University(Medical Science)

摘　　要：目的探讨气道受累的复发性多软骨炎（relapsing polyehondritis，RP）的临床特点。方法对5例气道受累的复发性多软骨炎患者的临床资料及相关检查进行分析，并结合文献复习。结果气道受累的RP临床表现为胸闷、气促、呼吸困难，咳嗽、咳痰、声嘶等。胸部CT检查早期可见气管、支气管壁弥漫性增厚伴管腔狭窄；疾病后期出现管腔重度狭窄，部分患者可有气道壁的钙化，气道吸气、呼气相明显动态狭窄。肺功能测定表现为不同程度的阻塞性通气功能障碍。气管镜检查发现气管-支气管软骨破坏而出现气道软骨环消失，管壁软化，呼气时部分气道陷闭。5例患者接受了糖皮质激素等治疗，其中2例患者症状明显改善，1例患者症状稍有改善，2例患者症状无改善。2例无改善患者中1例在给予气道金属支架置入后气道阻塞明显改善，1例拒绝金属支架置入等治疗。随访2-29个月后有3例存活，2例分别随访1个月，5个月后死亡。结论气道受累的RP是一种罕见疾病，诊断需结合临床表现、胸部CT和气管镜检查；皮质激素等治疗部分患者症状可显著改善，气管切开、气管支架置入、机械通气能缓解患者呼吸困难。气道受累的RP预后较差。Objective To study the clinical characteristics of relapsing polychondritis （RP） with the involvement of respiratory tract. Methods The clinical features, auxiliary tests and prognosis of RP with airway involvement in 5 patients admitted in our hospital since 2003 were analyzed retrospectively. Results The symptoms of RP in respiratory disorder included chest tight, dyspnea, breathless, cough, expectoration and hoarseness. Thoracic CT scan with three dimensional reconstruction of the airways demonstrated a diffusely thickened tracheobronchial wall with tracheobronchial stenosis in earlier period of the disease and showed severe narrowing or calcification of both trachea and main bronchi in later period. Spirometry showed severe obstructive ventilation disorder. Bronchoscopy revealed dynamic collapse of the proximal airways and dissolving of cartilage rings. In 5 patients, after initially receiving the medical drugs such as corticosteroids and immunosuppressive drugs, symptoms were improved significantly in 2 patients, mildly improved in 1 and progressed in 2 patients. One case relieved after receiving the self-expanding metallic stent replacement in the airways（ trachea and main bronchi）. The following-up period was 2 to 29 month,3 patients were still alive so far,2 cases of them died in the 1st and 5th months after the diagnosis respectively. Conclusion RP with respiratory tract involvement is a rare disease. This disease should be diagnosed with its clinical features, abnormal thoracic CT and bronchoscopic manifestation. Early stage diagnosis and therapy with corticosteroids, immunosuppressive agents could have a relative better prognosis, and in the later stage, invasive methods such as tracheotomy,tracheal stent and mechanical ventilation could soothe dyspnea.

关 键 词：多发性软骨炎 复发性 气道狭窄 诊断 

分 类 号：R56[医药卫生—呼吸系统]