Does Initial Progression of SSc-ILD Mean More Is to Come?

TOPLINE:

Interstitial lung disease (ILD) progression in patients with systemic sclerosis–associated ILD (SSc-ILD) did not predict subsequent progression at the next annual follow-up, with only 20% showing further progression. However, initial ILD progression was associated with an increased risk for mortality.

METHODOLOGY:

• Researchers conducted a multicenter observational study to evaluate whether an observed progression of ILD in patients with SSc-ILD leads to a risk for subsequent progression.
• They included 231 patients with SSc-ILD in the main cohort (mean age, 48 years; 76% women; mean disease duration, 10.4 years) from Oslo, Norway, and Zurich, Switzerland, enrolled between January 2001 and December 2019.
• Pulmonary function tests measuring forced vital capacity (FVC) and diffusing capacity for carbon monoxide were performed as per the American Thoracic Society–European Respiratory Society guidelines.
• ILD progression was defined as an absolute FVC decline of ≥ 5% over 12 months, with three alternative definitions of ILD progression — an absolute FVC decline of ≥ 10%, progressive pulmonary fibrosis, and progressive fibrosing ILD — all assessed at annual visits.
• Findings were validated in an enriched cohort comprising 68 patients with SSc-ILD from the main cohort and 53 patients from Michigan (n = 121; mean age, 52 years; 69% women; mean disease duration, 1.3 years). Furthermore, the effects of ILD progression on mortality were assessed.

TAKEAWAY:

• Among patients with initial ILD progression — an FVC decline of ≥ 5% from visit 1 to visit 2 — only 20% showed further progression at visit 3. However, among those without initial progression between visits 1 and 2, 31% showed progression between visits 2 and 3 (P = .048).
• Initial ILD progression with an FVC decline of ≥ 5% reduced the risk for subsequent progression (adjusted odds ratio [aOR], 0.28; P = .002; area under the curve, 0.71). Moreover, no risk was observed for subsequent progression using alternative definitions.
• Similar results were noted in the enriched cohort, with an FVC decline of ≥ 5% from visit 1 to visit 2 reducing the risk for subsequent progression from visit 2 to visit 3 (aOR, 0.22; P = .031).
• FVC decline ≥ 5% was associated with an increased risk for mortality in the main cohort (hazard ratio, 1.66; P = .030).

IN PRACTICE:

“These results challenge current treatment practices because, although initiating or escalating treatment should still be done in patients with ILD progression according to guidelines, our data suggest that treatment should be initiated or escalated in patients at risk of progression to prevent poor outcomes, although confirmation is needed to show a benefit of this strategy on improved outcome,” the authors wrote.

SOURCE:

The study was led by Anna-Maria Hoffmann-Vold, MD, Department of Rheumatology, Oslo University Hospital, Oslo, Norway. It was published online on May 14, 2025, in The Lancet Rheumatology. 

LIMITATIONS:

Only patients with consecutive lung function assessments were included, which may have enriched the sample with patients with severe or progressive ILD. Moreover, the findings may not be applicable to other types of ILD. Validation in more diverse populations may be needed as the study predominantly included White patients.

DISCLOSURES:

The study did not receive any specific funding. Several authors reported receiving speakers bureau and consulting fees, grants, and having other financial relationships with pharmaceutical, biotechnology, and other companies, including Boehringer Ingelheim, Janssen, Medscape, Genentech, Merck Sharp & Dohme, and Roche.

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