Cottin V, Nunes H, Brillet P-Y, Delaval P, Devouassoux G, Tillie-Leblond I, Israel-Biet D, Court-Fortune I, Valeyre D, Cordier J-F
Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires, Hôpital Cardiovasculaire et Pneumologique Louis Pradel, Université Claude Bernard, Lyon (Bron), France.
Eur Respir J. 2005 Oct;26(4):586-93. doi: 10.1183/09031936.05.00021005.
The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described. The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography. Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87% and finger clubbing in 43%. Pulmonary function tests were as follows (mean+/-sd): total lung capacity 88%+/-17, forced vital capacity (FVC) 88%+/-18, forced expiratory volume in one second (FEV1) 80%+/-21 (% predicted), FEV1/FVC 69%+/-13, carbon monoxide diffusion capacity of the lung 37%+/-16 (% predicted), carbon monoxide transfer coefficient 46%+/-19. Pulmonary hypertension was present in 47% of patients at diagnosis, and 55% during follow-up. Patients were followed for a mean of 2.1+/-2.8 yrs from diagnosis. Survival was 87.5% at 2 yrs and 54.6% at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival.
合并性肺纤维化和肺气肿所致综合征尚未得到全面描述。本文作者对61例胸部计算机断层扫描显示上叶肺气肿合并下叶弥漫性实质性肺疾病伴纤维化的患者进行了一项回顾性研究。患者（均为吸烟者）包括60名男性和1名女性，平均年龄65岁。所有患者均有劳力性呼吸困难。87%的患者可闻及基底啰音，43%的患者有杵状指。肺功能检查结果如下（均值±标准差）：肺总量88%±17，用力肺活量（FVC）88%±18，一秒用力呼气容积（FEV1）80%±21（预计值%），FEV1/FVC 69%±13，肺一氧化碳弥散量37%±16（预计值%），一氧化碳转运系数46%±19。47%的患者在诊断时存在肺动脉高压，随访期间这一比例为55%。患者从诊断开始平均随访2.1±2.8年。2年生存率为87.5%，5年生存率为54.6%，中位生存期为6.1年。诊断时存在肺动脉高压是预后的关键决定因素。作者据此将计算机断层扫描定义的合并性肺纤维化和肺气肿综合征个体化，其特征为肺量计检查结果低于正常、气体交换严重受损、肺动脉高压患病率高以及生存率低。
