• Clin Respir J · Apr 2018

    Meta Analysis

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

    • Martina Doubková, Jan Švancara, Michal Svoboda, Martina Šterclová, Vladimír Bartoš, Martina Plačková, Ladislav Lacina, Monika Žurková, Ilona Binková, Radka Bittenglová, Vladimíra Lošťáková, Zdeněk Merta, Lenka Šišková, Richard Tyl, Pavlína Lisá, Hana Šuldová, František Petřík, Jana Pšikalová, Vladimír Řihák, Tomáš Snížek, Pavel Reiterer, Jiří Homolka, Pavlína Musilová, Jaroslav Lněnička, Peter Palúch, Roman Hrdina, Renata Králová, Hana Hortvíková, Jana Strenková, and Martina Vašáková.
    • Department of Phthisiology Pulmonary Diseases and Tuberculosis, Masaryk University Faculty of Medicine and University Hospital, Brno, Czech Republic.
    • Clin Respir J. 2018 Apr 1; 12 (4): 1526-1535.

    IntroductionPrognostic 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.MethodsData of 514 subjects consecutively entered since 2012 into Czech EMPIRE IPF registry were analyzed.ResultsMedian 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.ConclusionsDLCO 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.© 2017 John Wiley & Sons Ltd.

      Pubmed     Full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…