Analysis of predictors of lung function decline in patients with systemic sclerosis associated ILD (SSc-ILD): data from the German Network for Systemic Sclerosis.

<bold>Background:</bold> The lung function decline in patients with systemic sclerosis SSc-ILD seems to be highly variable and is poorly understood. The study was aimed at identifying predictors of decline in FVC and DLco in patients with SSc-ILD. <bold>Patients and methods:</bold> Clinical data of 6,234 patients with SSc were collected in the German Network for Systemic Sclerosis registry. Disease progression was defined as ≥ 10% relative decline in FVC% pred. or ≥ 5 to < 10% decline in relative FVC pred. and 15% relative decline in DLCO% pred. from baseline. Change in FVC and DLco was analyzed using a mixed model for repeated measures (MMRM) with random imputation of missing data. Cox proportional hazards regression analysis was used to investigate predictors of lung function decline. <bold>Results:</bold> 1313 patients with SSc-ILD were included in the analysis. Mean follow-up time was 3.99 ± 1.79 years and 246 (19%) patients had ILD progression. Mean time to progression was 2.84 ± 1.72 years. The average rate of decline in FVC % pred. was 7.33% per year in patients with ILD progression with a difference of 16.91% to non-progressors (p=<0.001). The average rate of decline in DLco% pred. was 9.16% per year in patients with a difference of 12.09% to non-progressors (p= <0.001). Anti scl-70 and RNA polymerase III antibodies were the strongest predictors of ILD progression at Cox regression analysis (HR 1.916, CI: 1.002-3.955 and HR 2.898, CI: 1.370-4.739, p<0.001 and p=0.004, respectively). <bold>Conclusion:</bold> Disease progression in SSc-ILD patients seems to occur within the first 5 years after ILD diagnosis and to be strongly associated with autoantibody status.

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Published
2025-09-27
DOI
https://doi.org/10.1183/13993003.congress-2025.pa6120
Primary Topic
Systemic Sclerosis and Related Diseases
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article
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article

Analysis of predictors of lung function decline in patients with systemic sclerosis associated ILD (SSc-ILD): data from the German Network for Systemic Sclerosis.

Michael Kreuter, Jörg Henes, Gabriela Riemekasten, Gernot Keyßer et al.
Systemic Sclerosis and Related Diseases
article

Analysis of predictors of lung function decline in patients with systemic sclerosis associated ILD (SSc-ILD): data from the German Network for Systemic Sclerosis.

Michael Kreuter, Jörg Henes, Gabriela Riemekasten, Gernot Keyßer, Jörg H. W. Distler, Francesco Bonella, Claudia Günther, Margitta Worm, Ilona Jandova, Jan Ehrchen, G. Zeidler, Marc Schmalzing, Norbert Blank, Pia Moinzadeh, Alexander Kreuter, A. Alberding, Aaron Juche, Jithmi Weliwitage, N. Hunzelmann, Ulf Müller-Ladner
article en

Abstract

<bold>Background:</bold> The lung function decline in patients with systemic sclerosis SSc-ILD seems to be highly variable and is poorly understood. The study was aimed at identifying predictors of decline in FVC and DLco in patients with SSc-ILD. <bold>Patients and methods:</bold> Clinical data of 6,234 patients with SSc were collected in the German Network for Systemic Sclerosis registry. Disease progression was defined as ≥ 10% relative decline in FVC% pred. or ≥ 5 to < 10% decline in relative FVC pred. and 15% relative decline in DLCO% pred. from baseline. Change in FVC and DLco was analyzed using a mixed model for repeated measures (MMRM) with random imputation of missing data. Cox proportional hazards regression analysis was used to investigate predictors of lung function decline. <bold>Results:</bold> 1313 patients with SSc-ILD were included in the analysis. Mean follow-up time was 3.99 ± 1.79 years and 246 (19%) patients had ILD progression. Mean time to progression was 2.84 ± 1.72 years. The average rate of decline in FVC % pred. was 7.33% per year in patients with ILD progression with a difference of 16.91% to non-progressors (p=<0.001). The average rate of decline in DLco% pred. was 9.16% per year in patients with a difference of 12.09% to non-progressors (p= <0.001). Anti scl-70 and RNA polymerase III antibodies were the strongest predictors of ILD progression at Cox regression analysis (HR 1.916, CI: 1.002-3.955 and HR 2.898, CI: 1.370-4.739, p<0.001 and p=0.004, respectively). <bold>Conclusion:</bold> Disease progression in SSc-ILD patients seems to occur within the first 5 years after ILD diagnosis and to be strongly associated with autoantibody status.

University of Wuppertal (DE), Johannes Gutenberg University Mainz (DE), Heidelberg University (DE), University Medical Center Freiburg (DE), University Hospital Heidelberg (DE), Klinik Preetz (DE), Ruhrlandklinik (DE), Düsseldorf University Hospital (DE), University Medical Center of the Johannes Gutenberg University Mainz (DE), University Hospital Münster (DE), University Hospital Schleswig-Holstein (DE), Helios Universitätsklinikum Wuppertal (DE), HELIOS St. Elisabeth Klinik Oberhausen (DE), University Children's Hospital Tübingen (DE), Universitätsklinikum Würzburg (DE), Kerckhoff Klinik (DE), University Hospital in Halle (DE), University Hospital Cologne (DE), Immanuel Krankenhaus (DE), University Hospital Carl Gustav Carus (DE), Heinrich Heine University Düsseldorf (DE), Charité - Universitätsmedizin Berlin (DE), University of Tübingen (DE), University of Lübeck (DE)
Openalex Percentile: Top 50%
Systemic Sclerosis and Related Diseases
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