TOPLINE:

New-onset interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) occurred at a rate of 3.83 cases per 100 person-years, with detection noted up to 10 years post-negative screening at baseline. The incident ILD group had a higher risk for death than patients without lung involvement.

METHODOLOGY:

• Researchers analyzed data of 11,601 patients from the European Scleroderma Trials and Research group database (September 2003 to February 2022) who had available ILD status on high-resolution CT at baseline and during at least one follow-up.
• They divided patients into prevalent ILD (n = 6270) and ILD-negative (n = 5331) groups based on baseline high-resolution CT, with further classification of those in the ILD-negative group into incident ILD and ILD-negative groups during the follow-up.
• The primary outcome was the crude incidence of SSc-associated ILD.
• Secondary outcomes were identification of risk factors for new-onset ILD and its impacts on disease progression and mortality.

TAKEAWAY:

• Among the 5331 patients with SSc with negative baseline high-resolution CT, the incidence of new-onset ILD was 3.83 cases per 100 person-years, with continuous detection up to 10 years from baseline.
• Risk factors for new-onset ILD included a dyspnea New York Heart Association stage of 2 or higher (hazard ratio [HR], 1.15; 95% CI, 1.01-1.30), digital pitting scars (HR, 1.15; 95% CI, 1.01-1.31), muscle weakness (HR, 1.26; 95% CI, 1.06-1.49), increased inflammatory marker levels (HR, 1.42; 95% CI, 1.21-1.67), anti-topoisomerase antibody positivity (HR, 2.40; 95% CI, 2.03-2.82), and others.
• In patients with anti-topoisomerase antibody-negative status, a dyspnea New York Heart Association stage of 2 or higher (HR, 1.32; 95% CI, 1.11-1.58) and age (HR, 1.02; 95% CI, 1.01-1.02) were identified as independent predictors of new-onset ILD, whereas in patients with antibody-positive status, these factors were not independently linked to ILD onset.
• The risk for death was lower in both incident ILD (adjusted HR, 0.68; 95% CI, 0.51-0.89) and ILD-negative (adjusted HR, 0.35; 95% CI, 0.29-0.42) groups than in the prevalent ILD group. Moreover, the incident ILD group had a higher risk for death than the ILD-negative group (HR, 1.94; 95% CI, 1.41-2.67), even after adjustment for confounding factors.

IN PRACTICE:

“Our analysis also indirectly supports the rescreening for ILD in patients with an initially negative HRCT [high-resolution CT], as we observed patients undergoing more frequent HRCTs were detected with new-onset ILD almost 1 year earlier. This is an advance in the window of opportunity for the treatment of SSc-ILD, to possibly start treating earlier, as well as the basis for an enrichment cohort for preventive trials, in which pharmacologic and nonpharmacologic interventions might be tested,” the authors wrote.

SOURCE:

The study was led by Liubov Petelytska, MD, PhD, University Hospital Zurich, University of Zurich, Zurich, Switzerland. It was published online on January 17, 2026, in the Annals of the Rheumatic Diseases.

LIMITATIONS:

The long disease duration of participants could introduce bias. Not all patients with negative baseline scans underwent follow-up imaging, potentially leading to selection bias. The progression analysis focused on pulmonary function test deterioration and excluded the recent complex definition of progressive pulmonary fibrosis due to insufficient data in the dataset.

DISCLOSURES:

This study did not report any specific funding. Multiple authors disclosed receiving grants, fees, and research support from and having other ties with various pharmaceutical companies including Pfizer, Novartis, Boehringer Ingelheim, AstraZeneca, GlaxoSmithKline, Sanofi, and others. Two authors declared consultancy relationships and/or speaking engagements with Medscape, and one of them reported receiving speaker fees from Medscape.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.