Sobanski, V., de Vries-Bouwstra, J., Hoffmann-Vold, A.-M. et al. (102 more authors) (2025) Lung function and skin fibrosis changes as predictors of survival in SSc-associated interstitial lung disease: a EUSTAR study. Rheumatology. keaf264. ISSN 1462-0324 (In Press)
Abstract
Objectives
This study assessed how changes in lung function, skin fibrosis and digital ulceration (DU) burden predict mortality in patients with SSc-associated interstitial lung disease (SSc-ILD), the leading cause of death in SSc.
Methods
Adult SSc-ILD patients from the European Scleroderma Trials and Research (EUSTAR) database enrolled since January 2009 with a date of diagnosis, a follow-up visit for change evaluation within 12 months plus a further visit or mortality information were eligible. Twelve-month changes in lung function (per cent predicted forced vital capacity [FVC%pred] and diffusing capacity of the lungs for carbon monoxide [DLCO%pred]), modified Rodnan skin score (mRSS) and change in DU burden were assessed for associations with survival, using multivariable Cox regression analyses adjusted for age, sex, smoking status and immunosuppressive therapy.
Results
Of 893 SSc-ILD patients included, 94 (10.5%) died over a mean follow-up of 39.0 ± 23.9 months. Absolute deterioration in FVC >10%pred within 12 months (n = 78/638 evaluable) was predictive for decreased survival (hazard ratio [HR] 3.81; 95% CI 1.67–8.66), as were composite measures combining (i) >10% FVC decline or mRSS worsening (HR 2.82; 95% CI 1.43–5.56) and (ii) FVC decline ≥10% or 5–9% with DLCO decline ≥15% (HR 3.42; 95% CI 1.68–7.00), but not changes in DLCO, mRSS or DU burden alone.
Conclusions
Changes in lung function and skin fibrosis within 12 months should be considered when evaluating risk of mortality. The effect of pharmacological treatments aiming at stabilization of these variables should be evaluated prospectively in clinical trials.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. |
Keywords: | SSc-ILD, systemic sclerosis, interstitial lung disease, mortality, prognostic stratification |
Dates: |
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Institution: | The University of Leeds |
Academic Units: | The University of Leeds > Faculty of Medicine and Health (Leeds) > School of Medicine (Leeds) |
Depositing User: | Symplectic Publications |
Date Deposited: | 18 Jul 2025 11:44 |
Last Modified: | 18 Jul 2025 11:44 |
Status: | In Press |
Publisher: | Oxford University Press (OUP) |
Identification Number: | 10.1093/rheumatology/keaf264 |
Related URLs: | |
Sustainable Development Goals: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:229188 |