肺纤维化合并肺气肿综合征3例并文献复习  

Combined pulmonary fibrosis and emphysema syndrome：report of three cases and review of literature

作　　者：孙健[1] 沈巨信[1] 余月芳[1] 

机构地区：[1]绍兴市人民医院浙江大学绍兴医院呼吸,绍兴312000

出　　处：《国际呼吸杂志》2012年第12期891-895,共5页International Journal of Respiration

摘　　要：目的：探讨肺纤维化合并肺气肿综合征临床特点、诊断标准及影像学表现，并提高对其认识。方法分析2008年10月至2011年5月收治的3例肺纤维化合并肺气肿综合征病例临床特点、肺功能及影像学表现，并复习国内外文献。结果3例患者均为重度吸烟的老年男性，均以活动后呼吸困难为主要表现。胸部CT表现为下肺纤维化及上肺野肺气肿，肺功能表现为第1秒用力呼气容积、用力肺活量及肺总量轻度下降而一氧化碳弥散功能重度下降。其中2例合并肺动脉高压。经治疗后咳嗽及喘息症状能够缓解，但肺弥散功能改善不明显。结论诊断肺纤维化合并肺气肿综合征主要依赖胸部高分辨率CT，其特点为同时存在上肺野肺气肿和下肺野纤维化。肺功能表现为肺总量基本正常而弥散功能显著下降。临床治疗能部分缓解症状但难以改善肺弥散功能。肺纤维化合并肺气肿综合征发病机制、治疗及预后仍有待进一步研究。Objective To analyze clinical characteristics, diagnostic standard and radiological appearance in patients with combined pulmonary fibrosis and emphysema （CPFE） syndrome, and to improve awareness of it. Methods The clinical features, lung function and radiological imaging of three patients with CPFE syndrome since October 2008 to May 2011 were reported and the literatures were reviewed. Results All patients were older men and heavy smokers. Dyspnea on exertion was the main clinical features. HRCT findings included emphysema predominantly at the upper lobes, while reticular opacities,honeycombing were in the lower lobes. Pulmonary function demonstrated that forced expiratory volume in one second, forced vital capacity and total lung capacity were slightly lower, and the carbon monoxide diffusion capacity was significantly impaired. Pulmonary hypertension was seen in two patients. The symptoms of asthma and cough significantly relieved after treatment, but the carbon monoxide diffusion capacity was unimproved. Conclusions CPFE syndrome can be diagnosed according HRCT findings including co-existence of lower lung fibrosis and upper lung emphysema. Patients with CPFE syndrome can present with nearly normal total lung capacity but a remarkable impairment in pulmonary diffusion capacity. Treatment is effective for relieving clinical symptoms but not improving the lung diffusion capacity. Further studies to elucidate the pathogenesis and to explore the treatment and prognosis are warranted.

关 键 词：肺纤维化 肺气肿 肺纤维化合并肺气肿 

分 类 号：R563[医药卫生—呼吸系统]