Progression-survival dissociation in connective tissue disease (CTD)-associated interstitial lung disease (ILD): CTD-ILD versus non-CTD ILD in real-world practice
Year: 2026
Type: article
Study design: Observational Study
Abstract: Background Connective tissue disease-associated interstitial lung disease (CTD-ILD) is a clinically relevant subtype of fibrotic ILD. We compared baseline characteristics, treatment patterns, transplant-free survival (TFS) and progression-free survival (PFS) between CTD-ILD and non-CTD ILD. Methods In the prospective German INSIGHTS-ILD registry, patients with known CTD status at baseline were classified as CTD-ILD or non-CTD ILD. TFS and PFS were analysed using Kaplan–Meier and log-rank tests. PFS was defined by a prespecified composite end-point including lung function decline, oxygen escalation, 6-min walk distance decline, respiratory hospitalisation, death or clinical worsening prompting any medical therapy escalation or switch. Cox models included the unadjusted Model A; Model B adjusted for age, sex, smoking, comorbidity count and baseline antifibrotic therapy; Model C additionally adjusted for baseline forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide; and Model D additionally adjusted for time since diagnosis. Results 332 patients (35.7%) had CTD-ILD and 598 (64.3%) had non-CTD ILD. Patients with CTD-ILD were younger, more often female and more frequently received immunomodulatory therapy, whereas antifibrotic use was similar between groups. TFS was similar at 1 year (90.8% versus 90.9%; p=0.57) and at 2 years (83.7% versus 81.9%). In contrast, PFS was higher in CTD-ILD at 1 year (71.8% versus 62.7%; p=0.0032) and 2 years (52.6% versus 41.7%), with less FVC decline over follow-up. CTD-ILD was associated with a lower risk of progression in the unadjusted model (HR 0.73, 95% CI 0.59–0.90; p=0.003) and across all adjusted models. Conclusion In routine care, CTD-ILD showed similar TFS but better PFS and less FVC decline than non-CTD ILD.
Source: ERJ Open Research
Authors: Jürgen Behr, Francesco Bonella, Andreas Günther, Michael Kreuter, David Pittrow, Benjamin Seeliger, Antje Prasse, Christine Pausch, Dirk Skowasch, Heinrike Wilkens, Hubert Wirtz, Marlene Hechtner, Heike Biller, Jonas C. Schupp, Christian Grohé, Lars Hagmeyer, Stephan Budweiser, Ioana Andreica, Ulrich Neff, Sven Gläser, Martin Schwaiblmair, Peter Schramm, F. Joachim Meyer, Tobias Veit, Wolfgang Gesierich, Bernd Seese, Achim Grünewaldt, Philipp Markart, Michael Westhoff, Matthias Held, Joachim Kirschner, Julia Wälscher, Stephan Eisenmann, Stephan Walterspacher, Axel Kempa, Claus Neurohr, Claus-Peter Kreutz, Daniel Grund, Sabine Haberl, Ralf Ewert, Beate Stubbe, Markus Polke, Frank Reichenberger, Werner von Wulffen, Ekaterina Krauss, Michael Weber, Michael Dreher, Tim Oqueka, Maximilian Malfertheiner, Dirk Koschel, Martin Aringer, Silke Tello, Gernot Rohde, Wolfgang Alexander Seeger, Marion Frankenberger, Adrian Gillissen, Celine Vetter, Torsten Witte
Institutions: LMU Klinikum, Ludwig-Maximilians-Universität München, German Center for Lung Research, University Medical Center of the Johannes Gutenberg University Mainz, Technische Universität Dresden, Medizinische Hochschule Hannover, GWT-TUD GmbH (Germany), University Hospital Bonn, Universitätsklinikum des Saarlandes, University Hospital Leipzig, Boehringer Ingelheim (Germany), Evangelische Lungenklinik Berlin, Krankenhaus Bethanien, RoMed Kliniken, Rheumazentrum Ruhrgebiet, Ruhr University Bochum, Nuremberg Hospital, Vivantes Klinikum, University Hospital Augsburg, München Klinik Bogenhausen, München Klinik, Asklepios Fachkliniken München-Gauting, Klinikum Darmstadt, Klinikum Fulda, Lungenklinik Hemer, Klinikum Würzburg Mitte, University of Bamberg, Ruhrlandklinik, University of Duisburg-Essen, University Hospital in Halle, Klinikum Konstanz, Robert Bosch Hospital, Charité – Universitätsmedizin Berlin, Universitätsmedizin Greifswald, University Hospital Heidelberg, Justus-Liebig-Universität Gießen, Zentralklinik Bad Berka, Universitätsklinikum Aachen, RWTH Aachen University, University Medical Center Hamburg-Eppendorf, Klinik Donaustauf, University Hospital Carl Gustav Carus, Universitätsklinikum Gießen und Marburg, Goethe University Frankfurt, University Hospital Frankfurt
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $808
Funder: Boehringer Ingelheim
OpenAlex ID: W7214166221