Real-life characteristics and management of patients with fibrosing interstitial lung disease: INSIGHTS-ILD registry

Jürgen Behr¹, Michael Kreuter², Andreas U Guenther³, Francesco Bonella⁴, David Pittrow^{5

6}, Christine Pausch⁶, Benjamin Seeliger⁷, Antje Prasse⁷, Dirk Skowasch⁸, Henrike Wilkens⁹, Hans-Joachim Kabitz¹⁰, Hubert Wirtz¹¹, Martin Claussen¹², Christian Grohé¹³, Lars Hagmeyer¹⁴, Stephan Budweiser¹⁵, Ioana Andreica¹⁶, Ulrich Neff¹⁷, Heike Biller¹², Sven Glaeser¹⁸, Martin Schwaiblmair¹⁹, Peter Schramm²⁰, F Joachim Meyer²⁰, Karine Thabaret²⁰, Jens Klotsche²¹, Tobias Veit¹, Marion Frankenberger¹, Lea Drobbe¹⁸, Wolfgang Gesierich²², Bernd Seese²³, Elaine Koch²³, Achim Grünewaldt²⁴, Philipp Markart²⁵, Michael Westhoff²⁶, Matthias Held²⁷, Joachim Kirschner²⁸, Julia Wälscher⁵, Stephan Eisenmann²⁹, Claus Neurohr³⁰, Claus-Peter Kreutz²², Daniel Grund³¹, Sabine Haberl³², Ralf Ewert³³, Beate Stubbe³³, Markus Polke³⁴, Frank Reichenberger³⁵, Werner von Wulffen³⁵, Ekaterina Krauss⁴, Michael Weber³⁶, Stephan Walterspacher³⁷, Dirk Koschel³⁸

Affiliations

¹ Department of Medicine V, LMU University Hospital, LMU Munich, Comprehensive Pneumology Center, German Center for Lung Research (DZL), Munich, Germany.
² Mainz Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center and Department of Pulmonary, Critical Care and Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany.
³ Clinic for Pneumology, University of Giessen Lung Center, Giessen, Germany.
⁴ Pneumology Department, Center for Interstitial and Rare Lung Diseases, Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany.
⁵ Department for Clinical Pharmacology, Technical University, Dresden, Germany.
⁶ Innovation Center Real World Evidence, GWT-TUD GmbH, Dresden, Germany.
⁷ Pulmonology, Medical School, Hannover, Germany.
⁸ Internal Medicine II, University Hospital, Bonn, Germany.
⁹ Medical Clinic V, University Hospital, Bad Homburg, Germany.
¹⁰ Klinikum, Konstanz, Germany.
¹¹ University Hospital, Leipzig, Germany.
¹² LungenClinic, Grosshansdorf, Germany.
¹³ Clinic for Pneumology - ELK, Berlin, Germany.
¹⁴ Clinik for Pneumology and Allergology, Krankenhaus Bethanien, Solingen, Germany.
¹⁵ Medical Clinic III, RoMed Klinikum, Rosenheim, Germany.
¹⁶ Rheumazentrum Ruhrgebiet, Herne, Germany.
¹⁷ Klinikum, Nuernberg, Germany.
¹⁸ Clinic for Internal Medicine - Pneumology, Vivantes Hospital, Berlin Neukoelln, Germany.
¹⁹ Internal Medicine, University Hospital, Augsburg, Germany.
²⁰ Lungenzentrum München (Bogenhausen-Harlaching), München, Germany.
²¹ Epidemiology, German Rheuma Research Center (DRFZ), Berlin, Germany.
²² Asklepios Lungenklinik, Gauting, Germany.
²³ Thoraxzentrum Bezirk Unterfranken, Münnerstadt, Germany.
²⁴ Medical Clinic I, University Hospital, Frankfurt, Germany.
²⁵ Medizinische Klinik V, Herz-Thorax-Zentrum, Fulda, Germany.
²⁶ Zentrum für Pneumologie und Thoraxchirurgie, Lungenklinik, Hemer, Germany.
²⁷ Klinikum Würzburg Mitte - Missioklinik, Würzburg, Germany.
²⁸ CIMS Studienzentrum, Bamberg, Germany.
²⁹ U Klinik und Poliklinik für Innere Medizin I, Universitätsmedizin Halle, Universitätsklinikum, Halle, Germany.
³⁰ RBK Lungenzentrum, Stuttgart, Germany.
³¹ Charité - Universitätsmedizin, Berlin, Germany.
³² University Hospital, Augsburg, Germany.
³³ University Hospital, Greifswald, Germany.
³⁴ Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
³⁵ Augustinum, Lungenklinik, Munich, Germany.
³⁶ Zentralklinik Bad Berka, Bad Berka, Germany.
³⁷ Klinikum Konstanz und Lehrstuhl für Pneumologie, Universität Witten-Herdecke, Witten, Germany.
³⁸ Department of Internal Medicine and Pneumology, Fachkrankenhaus, Coswig, Medical Department I, Division of Pneumology, University Hospital Carl Gustav Carus, TU Dresden, Dresden, Germany.

PMID: 40491463
PMCID: PMC12147108
DOI: 10.1183/23120541.00926-2024

Real-life characteristics and management of patients with fibrosing interstitial lung disease: INSIGHTS-ILD registry

Jürgen Behr et al. ERJ Open Res. 2025.

. 2025 Jun 9;11(3):00926-2024.

doi: 10.1183/23120541.00926-2024. eCollection 2025 May.

Authors

Affiliations

¹ Department of Medicine V, LMU University Hospital, LMU Munich, Comprehensive Pneumology Center, German Center for Lung Research (DZL), Munich, Germany.
² Mainz Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center and Department of Pulmonary, Critical Care and Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany.
³ Clinic for Pneumology, University of Giessen Lung Center, Giessen, Germany.
⁴ Pneumology Department, Center for Interstitial and Rare Lung Diseases, Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany.
⁵ Department for Clinical Pharmacology, Technical University, Dresden, Germany.
⁶ Innovation Center Real World Evidence, GWT-TUD GmbH, Dresden, Germany.
⁷ Pulmonology, Medical School, Hannover, Germany.
⁸ Internal Medicine II, University Hospital, Bonn, Germany.
⁹ Medical Clinic V, University Hospital, Bad Homburg, Germany.
¹⁰ Klinikum, Konstanz, Germany.
¹¹ University Hospital, Leipzig, Germany.
¹² LungenClinic, Grosshansdorf, Germany.
¹³ Clinic for Pneumology - ELK, Berlin, Germany.
¹⁴ Clinik for Pneumology and Allergology, Krankenhaus Bethanien, Solingen, Germany.
¹⁵ Medical Clinic III, RoMed Klinikum, Rosenheim, Germany.
¹⁶ Rheumazentrum Ruhrgebiet, Herne, Germany.
¹⁷ Klinikum, Nuernberg, Germany.
¹⁸ Clinic for Internal Medicine - Pneumology, Vivantes Hospital, Berlin Neukoelln, Germany.
¹⁹ Internal Medicine, University Hospital, Augsburg, Germany.
²⁰ Lungenzentrum München (Bogenhausen-Harlaching), München, Germany.
²¹ Epidemiology, German Rheuma Research Center (DRFZ), Berlin, Germany.
²² Asklepios Lungenklinik, Gauting, Germany.
²³ Thoraxzentrum Bezirk Unterfranken, Münnerstadt, Germany.
²⁴ Medical Clinic I, University Hospital, Frankfurt, Germany.
²⁵ Medizinische Klinik V, Herz-Thorax-Zentrum, Fulda, Germany.
²⁶ Zentrum für Pneumologie und Thoraxchirurgie, Lungenklinik, Hemer, Germany.
²⁷ Klinikum Würzburg Mitte - Missioklinik, Würzburg, Germany.
²⁸ CIMS Studienzentrum, Bamberg, Germany.
²⁹ U Klinik und Poliklinik für Innere Medizin I, Universitätsmedizin Halle, Universitätsklinikum, Halle, Germany.
³⁰ RBK Lungenzentrum, Stuttgart, Germany.
³¹ Charité - Universitätsmedizin, Berlin, Germany.
³² University Hospital, Augsburg, Germany.
³³ University Hospital, Greifswald, Germany.
³⁴ Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
³⁵ Augustinum, Lungenklinik, Munich, Germany.
³⁶ Zentralklinik Bad Berka, Bad Berka, Germany.
³⁷ Klinikum Konstanz und Lehrstuhl für Pneumologie, Universität Witten-Herdecke, Witten, Germany.
³⁸ Department of Internal Medicine and Pneumology, Fachkrankenhaus, Coswig, Medical Department I, Division of Pneumology, University Hospital Carl Gustav Carus, TU Dresden, Dresden, Germany.

PMID: 40491463
PMCID: PMC12147108
DOI: 10.1183/23120541.00926-2024

Abstract

Background: This study aims to report real-life data on the characteristics and treatment patterns of patients with fibrosing interstitial lung disease (ILD; except idiopathic pulmonary fibrosis) across multiple specialised centres in Germany. Eligibility criteria included ILD affecting >10% of lung parenchyma on high-resolution computed tomography, a single breath diffusion capacity for carbon monoxide (D _LCO) ≤80% predicted and active treatment of lung disease.

Methods: As of the interim analysis cut-off, 655 patients (mean±sd age 65.9±11.7 years, 54.5% male) were included. The most common ILD subtypes were fibrosing hypersensitivity pneumonitis (31.2%), fibrosing ILD (22.0%), rheumatoid arthritis and connective tissue disease ILDs (13.0%) and unclassifiable fibrosing ILD (13.0%).

Results: Lung function metrics included total lung capacity at 68.3±17.6% predicted, forced vital capacity at 69.8±19.8% predicted, forced expiratory volume in 1 s at 73.7±19.5% predicted and D _LCO at 33.8±15.6% predicted. Current treatments included oral steroids (62.6%), antifibrotic therapy (50.7%), azathioprine (14.4%), methotrexate (10.2%) and mycophenolate mofetil (11.1%). Patients on antifibrotic therapy were typically older at diagnosis and registry inclusion, more often male, had more comorbidities, a lower 6-min walk distance and reduced lung function metrics compared with those not on antifibrotic therapy. Notably, 27.3% of the patients on antifibrotic therapy did not meet progression criteria (INBUILD), whereas 40.1% of patients not receiving antifibrotic therapy did meet those criteria.

Conclusion: The patient characteristics observed align with those observed in randomised controlled trials and other noninterventional studies. Patients on antifibrotic therapy generally had more severe disease profiles.

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Conflict of interest statement

Conflict of interest: J. Behr received honoraria for consulting and lectures from Actelion, AstraZeneca, BMS, Boehringer Ingelheim, Ferrer, Galapagos, Novartis, Roche and Sanofi-Genzyme. The INSIGHTS-ILD study is funded by Boehringer Ingelheim, Germany. Conflict of interest: M. Kreuter received honoraria for consulting and lectures from AstraZeneca, BMS, Boehringer Ingelheim, Ferrer, Galapagos and Roche. Conflict of interest: F. Bonella reports grants to his institution by Boehringer Ingelheim and Roche, and fees for consulting by Savara, Roche, Sanofi and Boehringer Ingelheim. Conflict of interest: D. Pittrow received consultancy fees from Alfasigma, Amgen, Aspen, Biogen, Daiichi Sankyo, Sanofi-Genzyme, Sandoz/Novartis and MSD. Conflict of interest: B. Seeliger received honoraria for lectures from Boehringer Ingelheim, AstraZeneca, Berlin-Chemie. Conflict of interest: S. Budweiser received honoraria for lectures and consulting from AstraZeneca, Boehringer Ingelheim, Berlin-Chemie, BMS, CSL Behring, Daiichi Sankyo, GSK, MSD, Novartis, Roche and Sanofi-Aventis. Conflict of interest: U. Neff reports lecture fees from Boehringer Ingelheim and Janssen-Cilag. Conflict of interest: F.J. Meyer received honoraria for lectures from Janssen-Cilag, Pfizer. Conflict of interest: A. Grünewaldt reports personal fees from Boehringer Ingelheim for lectures and GSK for advisory boards. Conflict of interest: P. Markart reports honoraria for consulting and lectures from Roche and Boehringer Ingelheim. Conflict of interest: R. Ewert has received speaker fees and fees for participation in advisory boards from United Therapeutics, OMT, Pfizer, GlaxoSmithKline (GSK), Actelion, Novartis, Bayer HealthCare and Encysive/Pfizer; grants from Actelion and Boehringer Ingelheim; and publication support and industry-sponsored grants from OMT, outside the submitted work. Conflict of interest: M. Polke received honoraria for consulting and lectures from AstraZeneca, Boehringer Ingelheim and Novartis. Conflict of interest: D. Koschel reports fees for consulting from Roche and Boehringer Ingelheim. Conflict of interest: The other authors declare no conflict of interest.

Figures

**FIGURE 2**
Affected lung parenchyma by percentage range in high-resolution computed tomography.

See this image and copyright information in PMC

References

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Real-life characteristics and management of patients with fibrosing interstitial lung disease: INSIGHTS-ILD registry

Affiliations

Real-life characteristics and management of patients with fibrosing interstitial lung disease: INSIGHTS-ILD registry

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

LinkOut - more resources

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Research Materials