Malli Foteini, Papakosta Despoina, Antoniou Katerina, Dimadi Maria, Polychronopoulos Vlassis, Malagari Katerina, Oikonomou Anastasia, Bouros Demosthenes E, Daniil Zoe
Respiratory Medicine Dept, University of Thessaly, School of Medicine, Larissa, Greece.
University of Thessaloniki, G. Papanikolaou Hospital, Dept of Pneumonology Thessaloniki, Thessaloniki, Greece.
ERJ Open Res. 2019 Mar 18;5(1). doi: 10.1183/23120541.00014-2018. eCollection 2019 Feb.
Combined pulmonary fibrosis and emphysema (CPFE) has recently received great attention, with studies suggesting that it presents a distinct clinical entity while others have challenged this hypothesis. This nationwide study aimed to describe a large cohort of Greek CPFE patients and to examine potential prognostic factors for survival.
This retrospective study included 97 patients with CPFE. Demographic and clinical data, pulmonary function tests, echocardiography results and bronchoalveolar lavage analysis were recorded.
Most patients were male (94.8%) and 92% were current or ex-smokers. Spirometry results were abnormal (forced vital capacity (FVC) 72.9±19.9% pred and forced expiratory volume in 1 s/FVC 82.9±9.7%) with reduced diffusing capacity of the lung for carbon monoxide ( ) (42.3±17.4% pred). Mean systolic pulmonary arterial pressure was 41.9±19.7 mmHg and pulmonary hypertension was present in 58.8% of patients. Mean 6-min walk distance was 335.4±159.4 m. Mean emphysema score was 14.23±8.69% and mean interstitial lung disease (ILD) extent was 39.58±19.82%. Mean survival was 84 months (95% CI 72-96 months). Patients with ≥39% pred had better survival than patients with <39% pred (p=0.031). Patients with ILD extent ≥30% had worse survival than patients with ILD extent <30% (p=0.037).
Our results indicate that CPFE patients have preserved lung volumes associated with disproportionately reduced , while reduced and increased ILD extent was associated with worse prognosis.
合并性肺纤维化和肺气肿（CPFE）最近受到了极大关注，研究表明它是一种独特的临床实体，而其他研究对这一假设提出了质疑。这项全国性研究旨在描述一大群希腊CPFE患者，并研究生存的潜在预后因素。
这项回顾性研究纳入了97例CPFE患者。记录了人口统计学和临床数据、肺功能测试、超声心动图结果和支气管肺泡灌洗分析。
大多数患者为男性（94.8%），92%为当前吸烟者或既往吸烟者。肺量计结果异常（用力肺活量（FVC）为预计值的72.9±19.9%，1秒用力呼气容积/FVC为82.9±9.7%），肺一氧化碳弥散量（ ）降低（为预计值的42.3±17.4%）。平均收缩期肺动脉压为41.9±19.7mmHg，58.8%的患者存在肺动脉高压。平均6分钟步行距离为335.4±159.4m。平均肺气肿评分为14.23±8.69%，平均间质性肺疾病（ILD）范围为39.58±19.82%。平均生存期为84个月（95%可信区间72 - 96个月）。 ≥预计值39%的患者比 <预计值39%的患者生存更好（p = 0.031）。ILD范围≥30%的患者比ILD范围<30%的患者生存更差（p = 0.037）。
我们的结果表明，CPFE患者的肺容积保留，但 不成比例地降低，而 降低和ILD范围增加与预后较差相关。
