特发性肺间质纤维化合并肺气肿与未合并肺气肿患者的临床对比研究

A comparison of clinical features between patients with idiopathic pulmonary fibrosis combined withemphysema and without emphysema

目的探讨特发性肺间质纤维化（IPF）合并肺气肿与未合并肺气肿患者的临床特点、肺功能、影像学及其预后的差异。方法从2002年1月至2007年1月在中国医科大学附属第一医院首次确诊为IPF的117例患者中筛选出符合肺纤维化合并肺气肿（CPFE）诊断的患者23例（CPFE组），选取2002-2003年确诊的单纯IPF患者33例作为对照组，回顾性分析两组患者的临床资料、肺功能、血氧分压、BALF细胞成分及生存时间的差异。结果（1）CPFE组吸烟指数为（28±18）包年，明显高于IPF组的（18±16）包年（t=2．10，P〈0．05）；两组性别、吸烟者比率及年龄差异无统计学意义。（2）CPFE组FEV1／FVC占预计值％为（77±7）％，明显低于IPF组的（83±8）％（t=3．55，P〈0．05）；CPFE组D1CO占预计值％为（44±12）％，明显低于IPF组的（54±16）％（t=2．48，P〈0．05）；CPFE组肺总量占预计值％明显高于IPF组[分别为（77±11）％和（64±12）％，t=3．93，P〈0．05]，肺活量及静息状态下血氧分压水平两组间无明显差异（均P〉0．05）。（3）两组间BALF细胞总数、巨噬细胞、中性粒细胞、淋巴细胞及嗜酸粒细胞比率无明显差异（均P〉0．05）。（4）两组中位生存时间差异无统计学意义[分别为（40±3）和（38±4）个月，X^2=0．65，P=0．79]。结论吸烟为IPF合并肺气肿的重要危险因素，IPF合并肺气肿患者的肺功能指标表现为肺容积轻度异常而弥散功能显著下降，合并肺气肿不影响IPF患者的预后。

Objective To investigate the differences of clinical features, pulmonary function tests, chest imaging and prognosis between patients with idiopathic pulmonary fibrosis （IPF） combined with emphysema and without emphysema. Methods Of the 117 patients diagnosed as IPF for the first time in the First Affiliated Hospital of China Medical University from January 2002 to January 2007, 23 cases met the diagnostic criteria for combined pulmonary fibrosis and emphysema （CPFE）. Thirty-three cases with isolated IPF from 2002 to 2003 were selected as the control group. Clinical characteristics, pulmonary function indexes, PaO2, bronchoalveolar lavage fluid （BALF） results and survival time were retrospectively analyzed and compared between the 2 groups. Results The smoking indexes （pack-years）were significantly higher in the CPFE group （ 28 ± 18 ） compared with the IPF group （ 18 ± 16）, t = 2. 10, P 〈 0. 05. No significant difference was observed between the 2 groups in terms of age, gender and the percentage of smokers. The CPFE group showed significantly lower FEV1/FVC% [ （77 ± 7 ） %, （ 83 ± 8 ） %, t = 3.55, P 〈 0. 05 ] and DLCO% pred [ （44 ± 12） %, （54 ± 16） %, t = 2.48, P 〈 0. 05 ] compared to those of the IPF group, while TLC% pred was significantly higher in the CPFE group [ （77 ± 11 ） %, （ 64 ± 12 ） %, t = 3.93, P 〈 0. 05 ]. VC% pred and PaO2 showed no significant difference between the 2 groups. The total cell count, the percentages of macrophages, neutrophils, lymphocytes and eosinophils in BALF showed no significant difference between the 2 groups. There was no significant difference in median survival time between the 2 groups. Conclusion Smoking was an important risk factor for IPF combined with emphysema. The pulmonary function of IPF combined with emphysema is characterized by a mild abnormality in the lung volume but a significant decrease in the diffusing capacity. Combined emphysema has no influence on the survival time of patients with IPF.