Predicting trajectories of lung function decline in systemic sclerosis–related interstitial lung disease
Date
2025
Authors
Zheng, B.
Nikpour, M.
Stevens, W.
Proudman, S.
Morrisroe, K.
Wang, M.
Man, A.
Baron, M.
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Journal article
Citation
Rheumatology, 2025; 64(11):5892-5898
Statement of Responsibility
Boyang Zheng, Mandana Nikpour, Wendy Stevens, Susanna Proudman, Kathleen Morrisroe, Mianbo Wang, Ada Man, Murray Baron
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Abstract
Objective SSc-related interstitial lung disease (SSc-ILD) is a major cause of morbidity. We aimed to identify patients following similar trajectories of forced vital capacity (FVC) decline, and examine their association with mortality and risk factors for FVC decline. Methods This is a multicentre retrospective study of 444 SSc patients with ILD and ≤7-year disease duration. Patients were grouped based on similar FVC decline trajectories using semi-parametric modelling with latent class analysis. Survival was compared between the worst FVC trajectory group and the others. Logistic regression models with backwards selection were applied to identify predictors of FVC trajectory using baseline disease features. Results Four FVC trajectory groups were identified. The most progressive trajectory declined by –2.18% per year and the other three trajectory groups were stable or progressed slowly. The most progressive group had a higher mortality rate than those with a stable/slow FVC trajectory (hazard ratio 2.95, 95% CI 1.74, 4.98). Baseline FVC (P < 0.001) and CRP elevation (P = 0.039) were associated the progressive trajectory. Baseline FVC ≤72% predicted the progressive trajectory with a sensitivity of 0.88 and specificity of 0.91. A lower baseline FVC was in turn associated with older age, Caucasian race, longer disease duration, anti-topoisomerase I presence and elevated CRP on exploratory analyses. Conclusion Distinct FVC trajectories are associated with different survival outcomes and the most important predictor of a progressive FVC trajectory was existing ILD severity. More work is needed to assess the utility of imaging or paraclinical findings that can improve prediction of distinct FVC trajectories.
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© The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.