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EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis

M. Doubková, J. Švancara, M. Svoboda, M. Šterclová, V. Bartoš, M. Plačková, L. Lacina, M. Žurková, I. Binková, R. Bittenglová, V. Lošťáková, Z. Merta, L. Šišková, R. Tyl, P. Lisá, H. Šuldová, F. Petřík, J. Pšikalová, V. Řihák, T. Snížek, P....

. 2018 ; 12 (4) : 1526-1535. [pub] 20170926

Language English Country England, Great Britain

Document type Journal Article, Meta-Analysis

INTRODUCTION: Prognostic factors of idiopathic pulmonary fibrosis (IPF) currently recognized include changes in vital capacity and radiologic findings. However, most of the prognostic studies in IPF are based on clinical studies with preselected IPF populations. Therefore, we decided to analyze the factors influencing IPF prognosis based on the real-practice data from our IPF registry. METHODS: Data of 514 subjects consecutively entered since 2012 into Czech EMPIRE IPF registry were analyzed. RESULTS: Median age of our patient cohort was 67 years (50-82). Median overall survival (OS) of the cohort was 63.1 months. The clinical course of IPF according to FVC (forced vital capacity) changes was stabilized in 32.8% of patients (29.7% according to DLCO [diffuse lung capacity] changes), slowly progressive in 39.5% (45%), rapidly progressive in 23.5% (20.7%); and 1.7% patients had at least one acute exacerbation during follow-up. Deterioration in FVC of ≥10% at month 12 and in DLCO of ≥15% at months 12, 18, and 24 influenced the OS significantly. The fast progressors defined by the DLCO decline rate had higher risk of death compared to those defined by the FVC change over time. In multivariate analysis, age ≥70 years, interstitial HRCT scores ≥3, and change in DLCO of ≥15% at month 12 were confirmed as factors negatively influencing OS. CONCLUSIONS: DLCO changes over time were shown as a better predictor of mortality compared with FVC changes in our study. In our opinion it is necessary to implement the DLCO analysis into clinical trials and routine practice.

1st Department of Tuberculosis and Respiratory Diseases General Hospital Prague and The 1st Medical Faculty of Charles University Czech Republic

Department of Phthisiology Pulmonary Diseases and Tuberculosis Masaryk University Faculty of Medicine and University Hospital Brno Czech Republic

Department of Pneumology 2nd Faculty of Medicine Charles University Prague and Motol University Hospital Prague Czech Republic

Department of Pneumology and Thoracic Surgery Hospital Na Bulovce Prague Czech Republic

Department of Pneumology Faculty of Medicine and Charles University Hradec Králové Czech Republic

Department of Pneumology University Hospital in Ostrava Faculty of Medicine Pilsen Charles University Czech Republic

Department of Pulmonary Diseases and Tuberculosis Masaryk Hospital Ústí nad Labem Czech Republic

Department of Respiratory Diseases Faculty of Medicine and Charles University Hospital Pilsen Czech Republic

Department of Respiratory Diseases Jihlava Hospital Czech Republic

Department of Respiratory Diseases Nový Jičín Hospital Czech Republic

Department of Respiratory Diseases Pardubice Hospital Czech Republic

Department of Respiratory Diseases Tomáš Baťa Regional Hospital Zlín Czech Republic

Department of Respiratory Diseases Znojmo Hospital Czech Republic

Department of Respiratory Medicine 1st Faculty of Medicine Charles University Thomayer Hospital Prague Czech Republic

Department of Respiratory Medicine Faculty of Medicine and Palacky University Hospital Olomouc Czech Republic

Institute of Biostatistics and Analyses Masaryk University Brno Czech Republic

PneumoAllergolog Department Kromeříž Hospital Czech Republic

Pulmonary Department České Budějovice Hospital Czech Republic

References provided by Crossref.org

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