目的探讨老年肺纤维化合并肺气肿(CPFE)综合征的临床特点。方法回顾性分析7例CPFE综合征患者的临床资料。结果 7例均为男性,年龄70-90岁;6例有重度吸烟史;均有活动后呼吸困难和双下肺爆裂音。胸部高分辨率CT检查显示双上肺为主的肺气肿及肺大疱形成,双下肺为主的胸膜下纤维条索、网格影及牵拉性支气管扩张。血气分析以低氧血症为主。5例有肺动脉高压。结论 CPFE综合征患者的临床特征为双上肺肺气肿合并双下肺纤维化病变,肺动脉高压发生率高,预后差。 Objective To investigate the clinical features of elderly pulmonary fibrosis complicated with emphysema (CPFE) syndrome. Methods The clinical data of 7 patients with CPFE syndrome were retrospectively analyzed. Results All the 7 patients were male, aged 70-90 years. Six patients had a history of heavy smoking. Both had dyspnea after exercise and double rupture of lungs. Chest high-resolution CT examination showed double upper lung-based emphysema and bulla formation, double lower lung-based subpleural fiber cords, grid shadow and traction bronchiectasis. Blood gas analysis mainly hypoxemia. 5 cases of pulmonary hypertension. Conclusions The clinical features of patients with CPFE syndrome are double upper pulmonary emphysema with double lower pulmonary fibrosis, high incidence of pulmonary hypertension and poor prognosis.