Jürgen Behr1,2,3,4, Antje Prasse4,5,6, Hubert Wirtz7, Dirk Koschel8, David Pittrow9,10, Matthias Held11, Jens Klotsche12, Stefan Andreas13, Martin Claussen4,14, Christian Grohé15, Henrike Wilkens16, Lars Hagmeyer17, Dirk Skowasch18, Joachim F Meyer19, Joachim Kirschner20, Sven Gläser21,22, Nicolas Kahn23, Tobias Welte4,5, Claus Neurohr24, Martin Schwaiblmair25, Thomas Bahmer14,26, Tim Oqueka27, Marion Frankenberger2, Michael Kreuter4,23. 1. Medizinische Klinik und Poliklinik V, LMU Klinikum, University of Munich, Munich, Germany juergen.behr@med.uni-muenchen.de. 2. Comprehensive Pneumology Center (CPC), Lungenforschungsambulanz, LMU Klinikum und Helmholtz Zentrum, Munich, Germany. 3. Asklepios Fachkliniken, München-Gauting, Germany. 4. German Center for Lung Research (DZL), Germany. 5. Klinik für Pneumologie, Medizinische Hochschule Hannover, Hannover, Germany. 6. Fraunhofer Institute ITEM, Hannover, Germany. 7. Abteilung für Pneumologie, Department Innere Medizin, Neurologie und Dermatologie, Universitätsklinikum Leipzig AöR, Leipzig, Germany. 8. Zentrum für Pneumologie, Fachkrankenhaus Coswig, Coswig, Germany. 9. Institut für Klinische Pharmakologie, Medizinische Fakultät, Technische Universität Dresden, Dresden, Germany. 10. GWT-TUD GmbH, Pharmacoepidemiology, Dresden, Germany. 11. Department of Internal Medicine, Respiratory Medicine and Ventilatory Support, Medical Mission Hospital, Central Clinic, Würzburg, Germany. 12. Epidemiologie, Deutsches Rheuma-Forschungszentrum, Berlin, Germany. 13. Lungenfachklinik Immenhausen und Universitätsmedizin Göttingen, Kardiologie und Pneumologie, Göttingen, Germany. 14. LungenClinic Grosshansdorf, Großhansdorf, Germany. 15. Klinik für Pneumologie - ELK, Berlin Buch, Berlin, Germany. 16. Klinik für Innere Medizin V, Pneumologie, Universitätsklinikum, Universitätskliniken des Saarlandes, Homburg, Germany. 17. Krankenhaus Bethanien, Klinik für Pneumologie und Allergologie, Zentrum für Schlaf- und Beatmungsmedizin; Institut für Pneumologie, Universität zu Köln, Solingen, Germany. 18. Medizinische Klinik und Poliklinik II, Universitätsklinikum, Bonn, Germany. 19. Lungenzentrum München, LZM Bogenhausen-Harlaching, Städtisches Klinikum München GmbH, Munich, Germany. 20. Center for Internal Medical Studies CIMS, Bamberg, Germany. 21. Klinik und Poliklinik für Innere Medizin B, Forschungsbereich Pneumologie und Pneumologische Epidemiologie, Universitätsmedizin Greifswald, Greifswald, Germany. 22. Klinik für Innere Medizin - Pneumologie und Infektiologie, Vivantes Klinikum Neukölln und Spandau, Berlin, Germany. 23. Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany. 24. Abteilung für Pneumologie und Beatmungsmedizin, Klinik Schillerhöhe, Gerlingen, Germany. 25. Medizinische Klinik, Universitätsklinikum, Augsburg, Germany. 26. Abteilung für Innere Medizin I, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Germany. 27. II Medizinische Klinik und Poliklinik, Universitätsklinikum, Hamburg-Eppendorf, Germany.
Abstract
OBJECTIVE: There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF). We aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions. METHODS: We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 interstitial lung disease expert centres in Germany. Data quality was ensured by automated plausibility checks, on-site monitoring, and source data verification. Propensity scores were applied to account for known differences in baseline characteristics between patients with and without antifibrotic therapy. RESULTS: Among the 588 patients suitable for analysis, the mean±sd age was 69.8±9.1 years, and 81.0% were male. The mean±sd duration of disease since diagnosis was 1.8±3.4 years. The mean±sd value at baseline for forced vital capacity (FVC) and diffusion capacity (D LCO) were 68.6±18.8% predicted and 37.8±18.5% predicted, respectively. During a mean±sd follow-up of 1.2±0.7 years, 194 (33.0%) patients died. The 1-year and 2-year survival rates were 87% versus 46% and 62% versus 21%, respectively, for patients with versus without antifibrotic therapy. The risk of death was 37% lower in patients with antifibrotic therapy (hazard ratio 0.63, 95% CI 0.45; 0.87; p=0.005). The results were robust (and remained statistically significant) on multivariable analysis. Overall decline of FVC and D LCO was slow and did not differ significantly between patients with or without antifibrotic therapy. CONCLUSIONS: Survival was significantly higher in IPF patients with antifibrotic therapy, but the course of lung function parameters was similar in patients with and without antifibrotic therapy. This suggests that in clinical practice, premature mortality of IPF patients eventually occurs despite stable measurements for FVC and D LCO.
OBJECTIVE: There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF). We aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions. METHODS: We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 interstitial lung disease expert centres in Germany. Data quality was ensured by automated plausibility checks, on-site monitoring, and source data verification. Propensity scores were applied to account for known differences in baseline characteristics between patients with and without antifibrotic therapy. RESULTS: Among the 588 patients suitable for analysis, the mean±sd age was 69.8±9.1 years, and 81.0% were male. The mean±sd duration of disease since diagnosis was 1.8±3.4 years. The mean±sd value at baseline for forced vital capacity (FVC) and diffusion capacity (D LCO) were 68.6±18.8% predicted and 37.8±18.5% predicted, respectively. During a mean±sd follow-up of 1.2±0.7 years, 194 (33.0%) patientsdied. The 1-year and 2-year survival rates were 87% versus 46% and 62% versus 21%, respectively, for patients with versus without antifibrotic therapy. The risk of death was 37% lower in patients with antifibrotic therapy (hazard ratio 0.63, 95% CI 0.45; 0.87; p=0.005). The results were robust (and remained statistically significant) on multivariable analysis. Overall decline of FVC and D LCO was slow and did not differ significantly between patients with or without antifibrotic therapy. CONCLUSIONS: Survival was significantly higher in IPF patients with antifibrotic therapy, but the course of lung function parameters was similar in patients with and without antifibrotic therapy. This suggests that in clinical practice, premature mortality of IPF patients eventually occurs despite stable measurements for FVC and D LCO.
Authors: M C Schimmelpennink; D B Meek; A D M Vorselaars; L C M Langezaal; C H M van Moorsel; J J van der Vis; M Veltkamp; J C Grutters Journal: Respir Res Date: 2022-06-25
Authors: Laurens J De Sadeleer; Stijn E Verleden; Jonas C Schupp; John E McDonough; Tinne Goos; Jonas Yserbyt; Elena Bargagli; Paola Rottoli; Naftali Kaminski; Antje Prasse; Wim A Wuyts Journal: Chest Date: 2022-01-19 Impact factor: 10.262
Authors: Jing Gao; Dimitrios Kalafatis; Lisa Carlson; Ida H A Pesonen; Chuan-Xing Li; Åsa Wheelock; Jesper M Magnusson; C Magnus Sköld Journal: Respir Res Date: 2021-02-05
Authors: Abigél Margit Kolonics-Farkas; Martina Šterclová; Nesrin Mogulkoc; Katarzyna Lewandowska; Veronika Müller; Marta Hájková; Mordechai Kramer; Dragana Jovanovic; Jasna Tekavec-Trkanjec; Michael Studnicka; Natalia Stoeva; Simona Littnerová; Martina Vašáková Journal: Front Med (Lausanne) Date: 2021-12-23
Authors: Detlef Kirsten; Ulrike de Vries; Ulrich Costabel; Dirk Koschel; Francesco Bonella; Andreas Günther; Jürgen Behr; Martin Claussen; Stefan Schwarz; Antje Prasse; Michael Kreuter Journal: Pneumologie Date: 2021-09-14
Authors: Manuel Röhrich; Dominik Leitz; Frederik M Glatting; Annika K Wefers; Oliver Weinheimer; Paul Flechsig; Nicolas Kahn; Marcus A Mall; Frederik L Giesel; Clemens Kratochwil; Peter E Huber; Andreas von Deimling; Claus Peter Heußel; Hans Ulrich Kauczor; Michael Kreuter; Uwe Haberkorn Journal: J Nucl Med Date: 2021-07-16 Impact factor: 11.082