肺纤维化合并肺气肿综合征5例并文献复习

Combined pulmonary fibrosis and emphysema： report of five cases and literature review

目的分析、总结肺纤维化合并肺气肿（CPFE）综合征患者的临床、肺功能及影像学特点。方法对我院2009年至2010年诊断的5例CPFE综合征患者的临床、肺功能及影像学等特点结合文献进行分析和总结。结果5例均为男性，年龄54～73岁，中位年龄63岁；4例有吸烟史，1例从事焊工30余年；5例均有活动后呼吸困难和双下肺爆裂音。胸部高分辨率CT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；肺功能结果[中位数（范围）]：用力肺活量（FVC）占预计值％为72％（71％～92％），第1秒用力呼气量（FEV，）／FVC为44％（28％～67％），FEV。占预计值％为36％（24％～83％），肺-氧化碳弥散量占预计值％为24％（16％～43％）。结论CPFE综合征患者的胸部高分辨率CT特点为双上肺野肺气肿合并双下肺野纤维化病变，肺功能的主要特点为弥散功能的显著降低而肺容积相对正常。其发病机制、治疗及预后等尚待进一步研究。

Objective To investigate the clinical, radiological features and pulmonary function changes of patients with combined pulmonary fibrosis and emphysema （CPFE） syndrome. Methods The clinical and radiological features and pulmonary function of five patients diagnosed with CPFE syndrome were retrospectively analyzed. Results All patients were male, 63 （54~73） years old, four of them were smokers, the other one had worked as a welder for more than 30 years. All patients had dyspnea on exertion and basal crackles could be heard at the lower fields of their both sides of lung. High resolution computerized tomography （HRCT） features included emphysema predominantly at the upper lobes, reticular opacities, honeycombing and traction bronchiectasis in the lower lobes. The pulmonary function demonstrated that FVC was 72% （71%~92%）, FEV1/FVC was 44%（28%~67%）, FEV, %pred was 36% （24% - 83%）, D1CO % pred was 24% （ 16% ~ 43%）. Conclusions Upper lobe emphysema combined with lower lobe fibrosis are the main HRCT features of CPFE syndrome patients. Remarkable impairment in gas exchange combined with normal or nearly normal lung volume can be observed in CPFE patients.