肺纤维化合并肺气肿综合征八例并文献复习

Combined pulmonary fibrosis and emphysema syndrome

目的 探讨肺纤维化合并肺气肿（CPFE）综合征的临床、肺功能及影像学特点.方法 结合文献对北京协和医院2006-2009年确诊的8例CPFE综合征患者的临床、肺功能及影像学特点进行分析.结果 8例均为男性,7例为吸烟者,年龄59～75岁,中位数为65岁,7例有活动后呼吸困难,4例有杵状指,6例有双下肺爆裂音.肺功能结果[中位数（范围）]：FVC占预计值%为73%（51%-92%）,FEV1/FVC为76%（60%-86%）,肺总量占预计值%为80%（59%～114%）,DLCO占预计值%为44%（16%-65%）.胸部高分辨率CT示双上肺为主的肺气肿及肺大疱,双下肺为主的网格影、蜂窝肺及牵张性支气管扩张.其中6例有肺动脉高压,肺动脉收缩压的中位数为49 mm Hg（1 mm Hg=0.133 kPa）,范围为35-101 mm Hg.结论 胸部高分辨率CT是诊断肺纤维化合并肺气肿综合征的重要手段,其特点为同时存在上肺野肺气肿和下肺野纤维化.肺功能表现为肺容积相对正常而弥散能力显著下降,是该综合征的突出特点.肺动脉高压发生率较高.肺纤维化合并肺气肿综合征能否作为一种独立的综合征,其发病机制、治疗及预后仍有待进一步研究.

Objective To retrospectively analyze clinical presentations, pulmonary function parameters and radiological appearance in patients with combined pulmonary fibrosis and emphysema （CPFE） syndrome. Methods We reviewed the clinical features, imaging, and lung function indices from 8 patients diagnosed with CPFE according to the findings by high resolution computerized tomography （HRCT） since 2006 to 2009 at Peking Union Medical College Hospital. Results All patients were male, aged 65 （59-75） years, and 7 of them were smokers. Dyspnea on exertion was presented in 7 patients. Basal crackles were heard in 6 patients and finger clubbing was observed in 4 patients. Pulmonary function demonstrated that forced expiratory volume in one second/forced vital capacity （FEV1/FVC） was slightly lower with a median of 76% （range 60% to 86% ） , forced vital capacity （FVC） was 73% （51% -92%）, and total lung capacity （TLC） was 80% （59% - 114%）. However, carbon monoxide diffusion capacity （DLCO） was significantly impaired （44% , 16% -65% ）. HRCT findings included emphysema predominantly at the upper zone, while reticular opacities, honeycombing and traction bronchiectasis were in the lower lobes. Pulmonary hypertension was seen in 6 patients. Conclusions The co-existence of lower lung fibrosis and upper lung emphysema was found in some smokers. Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange. A high prevalence of pulmonary hypertension is seen in CPFE syndrome. Further studies to elucidate the pathogenesis and to explore the treatment and prognosis are warranted.