累及气道的复发性多软骨炎的临床特征及其处理

目的提高对侵犯气管、支气管的复发性多软骨炎的临床特征及治疗方法的认识。方法回顾性分析2000年8月至2006年10月收治的13例侵犯气管、支气管的复发性多软骨炎患者的临床资料。结果13例累及气管、支气管的复发性多软骨炎患者中男9例，女4例，年龄30-61岁，平均年龄50岁。疾病早期临床表现以咳嗽、咽喉痛及声嘶多见，疾病后期常表现为胸闷、气促、呼吸困难，重者可表现为下呼吸道反复感染和（或）呼吸衰竭。支气管镜检查早期可见喉、声带水肿，气管、支气管黏膜充血、水肿，软骨环肿胀及管腔狭窄；疾病后期由于气管、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。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。肺功能测定表现为不同程度的阻塞性通气功能障碍。13例患者中有12例患者接受了糖皮质激素和（或）免疫抑制剂治疗，其中6例疾病早期患者症状均有不同程度的改善，其余6例患者症状无改善，在给予气道金属支架置入后（共放置气管、支气管支架12枚）5例患者气道阻塞明显改善，1例无缓解，仍需呼吸机辅助通气。随访1—48个月后有12例存活，1例随访2年后死亡。结论侵犯气管、支气管的复发性多软骨炎患者疾病早期糖皮质激素治疗可显著改善症状，疾病后期出现明显气道阻塞时可行支架置入、气管切开及呼吸机辅助通气治疗，以延长生存期。

Clinical features and treatment of relapsing polychondritis with involvement of the respiratory tractreport of thirteen cases

OBJECTIVE: To describe the clinical features and treatment of relapsing polychondritis with involvement of the respiratory tract. METHOD: Thirteen cases (admitted from Aug 2000 to Oct 2006) of relapsing polychondritis with involvement of the respiratory tract treated in our hospital were retrospectively analyzed. RESULTS: There were 9 males and 4 females, with ages ranging from 30 to 61 years (mean 50 years). At early stage of the disease, clinical manifestations included cough, throat pain and hoarseness. Patients in later stage usually complained of chest distress, shortness of breath and dyspnea. Severe complications were repeated lower respiratory tract infections and/or respiratory failure. Bronchoscopic examination revealed an edematous larynx, narrowing of the glottis, tracheobronchial edema, turgescence of bronchial cartilage rings and airway stenosis at early stage. At later stage of the disease, malacia of trachea and bronchi due to disappearance of bronchial cartilage rings, and partly obliteration of the both trachea and main bronchus on expiration were demonstrated. 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 of both trachea and main bronchi in later period. Lung function measurements showed a moderate obstructive ventilatory disorder in 5 patients. Medical treatment with corticosteroids and immunosuppressive drugs was given in 12 patients. Symptoms were improved in 6 patients in earlier period of the disease, however, 6 patients in later period were not relieved. Twelve self-expanding metallic stents were placed in the airways (trachea and/or main bronchi), and obstruction of the respiratory tract was relieved in 5 patients, but there was no improvement in 1 patient who later was treated with positive airway pressure support. The 13 patients were followed for 1 to 48 months, and 12 patients survived, but one patient died 2 years after diagnosis. CONCLUSIONS: Corticosteroid therapy is effective in improving the symptoms and delaying the progression of relapsing polychondritis with involvement of the respiratory tract at early stage. At later stage of the disease, airway interventional therapy, such as metallic stent placement, tracheostomy or positive airway pressure support, can be used to treat airway obstruction and to improve the survival.