目的探讨混合性结缔组织病(MCTD)相关性肺部病变的临床特点、诊断、治疗方法及预后。方法对哈尔滨医科大学附属第二医院呼吸科1996年1月至2008年6月入院治疗的112例诊断明确的MCTD患者进行回顾性分析,对其临床表现、放射学、心脏彩超及肺功能进行观察。对其中44例患者进行随访。结果112例MCTD患者中肺部有病变者48例(42.8%),其中女43例。主要症状是气短(11.6%)、胸痛(3.6%)和咳嗽(3.6%)。48例肺部存在病变患者中,25例患者肺功能下降和超声影像学异常时可无任何呼吸系统症状(52.1%)。98例MTCD患者中,影像学异常者41例(41.8%),常见的是胸腔积液15例(36.6%)和胸膜肥厚6例(14.6%)。45例行肺功能检查30例异常,其中一氧化碳弥散量(DLCO)异常22例(73.3%)。78例行超声心动图检查,发生肺动脉高压(PAH)者8例(10.3%)。随访7例发生PAH患者,3例死亡。结论MCTD肺部病变不少见,严重影响预后,DLCO是最敏感的评价MCTD患者肺功能下降的参数,PAH是混合性结缔组织病最常见的死亡原因。 Objective To investigate the clinical features, diagnosis, treatment and prognosis of pulmonary lesions associated with mixed connective tissue disease (MCTD). Methods One hundred and twelve cases of diagnosed MCTD admitted to Department of Respiratory, Second Affiliated Hospital of Harbin Medical University from January 1996 to June 2008 were retrospectively analyzed, and their clinical manifestations, radiology, echocardiography and pulmonary function were observed . Forty-four patients were followed up. Results Among the 112 patients with MCTD, there were 48 patients (42.8%) with lesions in the lungs, including 43 females. The main symptoms are shortness of breath (11.6%), chest pain (3.6%) and cough (3.6%). Among the 48 patients with pathological changes in the lungs, 25 patients had no respiratory symptoms (52.1%) with decreased lung function and abnormal ultrasound findings. Among the 98 patients with MTCD, 41 (41.8%) had abnormal imaging, 15 (36.6%) had hydrothorax and 6 (14.6%) had pleural hypertrophy. Thirty-five cases of abnormal lung function tests were performed in 45 cases, of which 22 cases (73.3%) had abnormal carbon monoxide dispersion (DLCO). 78 cases of echocardiography, pulmonary hypertension (PAH) in 8 cases (10.3%). Seven PAH patients were followed up and 3 died. Conclusions It is not uncommon for MCTD pulmonary lesions to affect prognosis seriously. DLCO is the most sensitive parameter to evaluate pulmonary function decline in patients with MCTD. PAH is the most common cause of death in mixed connective tissue diseases.