Objectives This study aimed to describe the clinical characteristics of patients with progressive pulmonary fibrosis (PPF) in systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and to explore factors associated with PPF. Methods Medical records of patients with SSc who visited Seoul St. Mary’s Hospital between April 2015 and March 2025 were retrospectively reviewed. PPF in SSc-ILD was defined as a relative decline in predicted forced vital capacity (FVC) ≥ 10% within 24 months. Clinical characteristics and outcomes were compared using logistic regression and survival analyses. Results Among 319 patients with SSc evaluated with thoracic CT, ILD was identified in 168 (52.7%). Of the 140 patients with SSc-ILD who had serial spirometry, 27 (19.3%) developed PPF. Patients with PPF exhibited digital gangrene (25.9% vs. 8.8%, P = 0.02), pulmonary hypertension (40.7% vs. 20.0%, P = 0.04), and mixed NSIP–UIP (11.1% vs. 1.8%, P = 0.049) more frequently than those without PPF. Treatment with methotrexate (37.0% vs. 15.0%, P = 0.01) and nintedanib (18.5% vs. 1.8%, P = 0.003) was also more prevalent. None of the variables remained statistically significant in the multivariable analysis, and overall survival did not differ between patients with and without PPF. Conclusion In patients with SSc-associated ILD, PPF was associated with prominent vascular manifestations and mixed NSIP–UIP. These findings underscore the heterogeneity of SSc-ILD and highlight the importance of close and longitudinal pulmonary function monitoring to identify disease progression. <Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> Key Points • ILD is common in systemic sclerosis, and a subset of patients with SSc-ILD develop PPF. • Patients with PPF more frequently exhibit vascular manifestations and distinct radiologic features, including mixed NSIP–UIP. • Regular longitudinal monitoring with pulmonary function tests is essential for early detection of disease progression in SSc-ILD. </entry> </row> </tbody> </tgroup> </Table>

Clinical characteristics and factors associated with progressive pulmonary fibrosis in patients with systemic sclerosis-associated interstitial lung disease

期刊论文
发表日期：

2026年7月13日

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Hee Won Park,
Youngjae Park,
Jennifer Jooha Lee,
Ji Hyeon Ju,
Wan-Uk Kim,
Sung-Hwan Park,
Seung-Ki Kwok

摘要

Objectives

This study aimed to describe the clinical characteristics of patients with progressive pulmonary fibrosis (PPF) in systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and to explore factors associated with PPF.

Methods

Medical records of patients with SSc who visited Seoul St. Mary’s Hospital between April 2015 and March 2025 were retrospectively reviewed. PPF in SSc-ILD was defined as a relative decline in predicted forced vital capacity (FVC) ≥ 10% within 24 months. Clinical characteristics and outcomes were compared using logistic regression and survival analyses.

Results

Among 319 patients with SSc evaluated with thoracic CT, ILD was identified in 168 (52.7%). Of the 140 patients with SSc-ILD who had serial spirometry, 27 (19.3%) developed PPF. Patients with PPF exhibited digital gangrene (25.9% vs. 8.8%, P = 0.02), pulmonary hypertension (40.7% vs. 20.0%, P = 0.04), and mixed NSIP–UIP (11.1% vs. 1.8%, P = 0.049) more frequently than those without PPF. Treatment with methotrexate (37.0% vs. 15.0%, P = 0.01) and nintedanib (18.5% vs. 1.8%, P = 0.003) was also more prevalent. None of the variables remained statistically significant in the multivariable analysis, and overall survival did not differ between patients with and without PPF.

Conclusion

In patients with SSc-associated ILD, PPF was associated with prominent vascular manifestations and mixed NSIP–UIP. These findings underscore the heterogeneity of SSc-ILD and highlight the importance of close and longitudinal pulmonary function monitoring to identify disease progression.

Key Points

• ILD is common in systemic sclerosis, and a subset of patients with SSc-ILD develop PPF.

• Patients with PPF more frequently exhibit vascular manifestations and distinct radiologic features, including mixed NSIP–UIP.

• Regular longitudinal monitoring with pulmonary function tests is essential for early detection of disease progression in SSc-ILD.