Clin Rheumatol. 2026 Jul 13. doi: 10.1007/s10067-026-08131-w. Online ahead of print.
ABSTRACT
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.
PMID:42440242 | DOI:10.1007/s10067-026-08131-w
