Martina Doubková1, Jan Švancara2, Michal Svoboda2, Martina Šterclová3, Vladimír Bartoš4, Martina Plačková5, Ladislav Lacina6, Monika Žurková7, Ilona Binková1, Radka Bittenglová8, Vladimíra Lošťáková7, Zdeněk Merta1, Lenka Šišková9, Richard Tyl10, Pavlína Lisá11, Hana Šuldová12, František Petřík11, Jana Pšikalová13, Vladimír Řihák9, Tomáš Snížek14, Pavel Reiterer15, Jiří Homolka16, Pavlína Musilová14, Jaroslav Lněnička15, Peter Palúch3, Roman Hrdina17, Renata Králová18, Hana Hortvíková5, Jana Strenková2, Martina Vašáková3. 1. Department of Phthisiology Pulmonary Diseases and Tuberculosis, Masaryk University Faculty of Medicine and University Hospital, Brno, Czech Republic. 2. Institute of Biostatistics and Analyses, Masaryk University, Brno, Czech Republic. 3. Department of Respiratory Medicine, First Faculty of Medicine, Charles University, Thomayer Hospital, Prague, Czech Republic. 4. Department of Pneumology, Faculty of Medicine and Charles University, Hradec Králové, Czech Republic. 5. Department of Pneumology, University Hospital in Ostrava, Faculty of Medicine, Pilsen, Charles University, Czech Republic. 6. Department of Pneumology and Thoracic Surgery, Hospital Na Bulovce, Prague, Czech Republic. 7. Department of Respiratory Medicine, Faculty of Medicine and Palacky University Hospital Olomouc, Czech Republic. 8. Department of Respiratory Diseases, Faculty of Medicine and Charles University Hospital Pilsen, Czech Republic. 9. Department of Respiratory Diseases, Tomáš Baťa Regional Hospital, Zlín, Czech Republic. 10. Department of Respiratory Diseases, Nový Jičín Hospital, Czech Republic. 11. Department of Pneumology, Second Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic. 12. Pulmonary Department, České Budějovice Hospital, Czech Republic. 13. PneumoAllergolog Department, Kromeříž Hospital, Czech Republic. 14. Department of Respiratory Diseases, Jihlava Hospital, Czech Republic. 15. Department of Pulmonary Diseases and Tuberculosis, Masaryk Hospital, Ústí nad Labem, Czech Republic. 16. First Department of Tuberculosis and Respiratory Diseases, General Hospital in Prague and The First Medical Faculty of Charles University, Czech Republic. 17. Department of Respiratory Diseases, Znojmo Hospital, Czech Republic. 18. Department of Respiratory Diseases, Pardubice Hospital, Czech Republic.
Abstract
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.
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.
Authors: Martina Doubkova; Eva Kriegova; Simona Littnerova; Petra Schneiderova; Martina Sterclova; Vladimir Bartos; Martina Plackova; Monika Zurkova; Radka Bittenglova; Vladimira Lostaková; Lenka Siskova; Pavlina Lisa; Hana Suldova; Michael Doubek; Jana Psikalova; Tomas Snizek; Pavlina Musilova; Martina Vasakova Journal: Ther Adv Respir Dis Date: 2021 Jan-Dec Impact factor: 4.031
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Authors: Laurie D Snyder; Christopher Mosher; Colin H Holtze; Lisa H Lancaster; Kevin R Flaherty; Imre Noth; Megan L Neely; Anne S Hellkamp; Shaun Bender; Craig S Conoscenti; Joao A de Andrade; Timothy Pm Whelan Journal: BMJ Open Respir Res Date: 2020-07
Authors: Daniel A Culver; Jürgen Behr; John A Belperio; Tamera J Corte; Joao A de Andrade; Kevin R Flaherty; Mridu Gulati; Tristan J Huie; Lisa H Lancaster; Jesse Roman; Christopher J Ryerson; Hyun J Kim Journal: Am J Respir Crit Care Med Date: 2019-07-15 Impact factor: 21.405