Progression-survival dissociation in connective tissue disease (CTD)-associated interstitial lung disease (ILD): CTD-ILD versus non-CTD ILD in real-world practice

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.

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Publication Details

Journal
ERJ Open Research
Published
2026-09-24
DOI
https://doi.org/10.1183/23120541.00788-2026
Primary Topic
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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article
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article

Progression-survival dissociation in connective tissue disease (CTD)-associated interstitial lung disease (ILD): CTD-ILD versus non-CTD ILD in real-world practice

Heinrike Wilkens, Gernot G. U. Rohde, Adrian Gillissen, Markus Polke et al.
ERJ Open Research
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
article

Progression-survival dissociation in connective tissue disease (CTD)-associated interstitial lung disease (ILD): CTD-ILD versus non-CTD ILD in real-world practice

Heinrike Wilkens, Gernot G. U. Rohde, Adrian Gillissen, Markus Polke, Jonas C. Schupp, Stephan Eisenmann, Stephan Budweiser, Beate Stubbe, Peter Schramm, Ioana Andreica, Tim Oqueka, Céline Vetter, Christine Pausch, Benjamin Seeliger, Hubert Wirtz, David Pittrow, Antje Prasse, Tobias Veit, Lars Hagmeyer, Wolfgang Gesierich, Ekaterina Krauss, Marion Frankenberger, Daniel Grund, Francesco Bonella, Achim Grünewaldt, Stephan Walterspacher, Michael Westhoff, J Kirschner, Bernd Seese, Claus-Peter Kreutz, Matthias Held, Sabine Haberl, Frank Reichenberger, Julia Wälscher, Silke Tello, Heike Biller, Claus Neurohr, Philipp Markart, Wolfgang Alexander Seeger, F. Joachim Meyer, Michael Dreher, Ulrich Neff, Christian Grohé, Maximilian Malfertheiner, Andreas Günther, Werner von Wulffen, Axel Kempa, Marlene Hechtner, Michael Weber, Jürgen Behr, Martin Schwaiblmair, Martin Aringer, Dirk Skowasch, Ralf Ewert, Torsten Witte, Sven Gläser, Michael Kreuter, Dirk Koschel
article en

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.

ERJ Open Research
Good health and well-being
Openalex Percentile: Top 12%
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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