@article{3106274,
    title = "Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort",
    author = "Malli, F. and Papakosta, D. and Antoniou, K. and Dimadi, M. and Polychronopoulos, V. and Malagari, K. and Oikonomou, A. and Bouros, D.E. and Daniil, Z.",
    journal = "ERJ Open Research",
    year = "2019",
    volume = "5",
    number = "1",
    publisher = "European Respiratory Society",
    issn = "2312-0541",
    doi = "10.1183/23120541.00014-2018",
    keywords = "adult;  Article;  cohort analysis;  disease severity;  Doppler echocardiography;  emphysema;  female;  forced expiratory volume;  forced vital capacity;  human;  lung fibrosis;  lung function test;  lung lavage;  major clinical study;  male;  middle aged;  mortality;  multicenter study;  overall survival;  pulmonary hypertension;  retrospective study;  six minute walk test;  spirometry;  systolic blood pressure",
    abstract = "Background: 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. Methods: This retrospective study included 97 patients with CPFE. Demographic and clinical data, pulmonary function tests, echocardiography results and bronchoalveolar lavage analysis were recorded. Results: 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 (DLCO) (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 DLCO ≥39% pred had better survival than patients with DLCO <39% pred (p=0.031). Patients with ILD extent ≥30% had worse survival than patients with ILD extent <30% (p=0.037). Conclusions: Our results indicate that CPFE patients have preserved lung volumes associated with disproportionately reduced DLCO, while reduced DLCO and increased ILD extent was associated with worse prognosis. © ERS 2019."
}