特发性肺间质纤维化合并肺气肿的临床特征分析

目的探讨特发性肺问质纤维化（IPF）合并肺气肿（CPFE）的临床特点及意义，提高对本疾病的认识和诊断水平。方法选择31例IPF合并CPFE的患者和36例单纯IPF患者作为研究对象，回顾性分析两组患者临床资料、肺功能、血气分析、胸部高分辨CT（HRCT）表现及生存时间差异。结果两组性别、年龄、吸烟者比例无显著差异，CPFE组吸烟指数为（38．1±9．0）年包，明显高于IPF组。CPFE组患者用力肺活量（FVC）占预计值％为（78．6±11．2）％、第1秒用力吸气容积FEV1／FVC为（69．1±6．0）％、一氧化碳弥散量（DLCO）占预计值％为（32．1±11．8）％，均明显低于IPF组，CPFE组患者肺总量（TLC）占预计值％为（76．3±8．1）％，VC占预计值％为（70．7±11．6）％，均明显高于IPF组。CPFE组患者肺动脉收缩压（CSPA）为（59．6±20．3）mmHg，明显高于IPF组。静息吸气状态下，CPFE组患者PaO2水平为（50．3±5．9）mmHg，明显低于IPF组。CPFE组患者胸部HRCT主要表现为双上肺野肺气肿和肺大疱，双下肺野呈网格影、蜂窝肺和牵张性支气管扩张。两组中位生存时间无统计学差异[分别为（38±5）和（41±4）个月，Х^2=0．55，P=0．63]。结论吸烟为CPFE的重要危险因素，当CPFE时，肺功能、血气分析、HRCT表现存在与单纯IPF不同的特点，与单纯IPF比较，预后无明显差异。

Clinical characteristics of idiopathic pulmonary fibrosis combined with emphysema

Objective To study the clinical characteristics and clinical significance of idiopathic pulmonary fibrosis （IPF） combined with emphysema （CPFE） in order to improve the understanding and the diagnostic ability. Methods A total of 31 patients with CPFE and 36 patients with IPF were enrolled in the retrospective study. Clinical characteristics were compared between the two groups, including general cliniacal data, pulmonary function test, blood-gas analysis, high resolution computed tomography （HRCT） and the survival time. Results No significant difference was observed between the two groups in terms of gender, age and percentage of smokers. The smoking indexes （pack-years） were significantly higher in the CPFE group （38.1 ± 9. 0） compared with the IPF group. The CPFE group showed significantly lower FVC% pred [（78.6±11.2）%], FEV1/FVC[（69. 1 ±6.0）%± and DLCO% pred[（32. 1 ±11.8）%] compared to those of the IPF group, while TLC%pred [（76.3 ±8. 1）%] and VC% pred[（70.7 ± 11.6）%1 were significantly higher in the CPFE group. The SPAP was significantly higher in the CPFE group[（59.6 ±20.3 ） mmHg], while PaO2 was significantly lower in the CPFE group [ （50.3 ± 5.9 ） mmHg 1- HRCT findings of the CPFE group included emphysema predominantly at the upper zone, while reticular opacities, honeycombing and traction bronchiectasis were in the lower lobes. There was no significant difference in median survival time between the two groups[ （38 ±5）months,（41 ±4）months,Х^2 =0.55, P =0.631. Conclusions Smoking is an important risk factor for CPFE. IPF and emphysema can coexist in some patients, the presentations of pulmonary function, blood-gas analysis, HRCT are markely different from patients with IPF alone, but no difference in survival compared with IPF alone.