Uneven Disease Course Requires Careful Monitoring of Interstitial Lung Disease

Although about 30% of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) showed progression of interstitial lung disease during any 12-month span, two-thirds of patients with SSc-ILD showed progression at some point over a 5-year period, according to a study published in the Annals of the Rheumatic Diseases.

“SSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important,” wrote researchers. “Novel treatment concepts, with treatment initiation before FVC [forced vital capacity] decline occurs, should aim for prevention of progression to avoid irreversible organ damage.”

The study focused on patients with SSc-ILD in the European Scleroderma Trials And Research (EUSTAR) database. Researchers were interested in the overall disease course and progression patterns of ILD in the patient population.

Among 826 patients with SSc-ILD, FVC measurements showed that 27% experienced progressive ILD over a period of 12±3 months, according to the study.

When researchers focused on 535 patients with multiple FVC measurements over an average 5 years of follow-up, they found that between 23% and 27% of patients showed progressive ILD in each 12-month period, yet many did not show progression in consecutive periods.

“Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline,” researchers reported, “whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline.”

The strongest predictors of 5-year FVC decline were male sex, skin fibrosis, and the presence of reflux/dysphagia symptoms. Other predictors included the presence of inflammation and shorter disease duration.

“These parameters may also be applied in daily clinical practice,” researchers wrote, “helping to identify patients who should receive treatment early, even before the first FVC decline has occurred.”

—Jolynn Tumolo

Reference

Hoffmann-Vold AM, Allanore Y, Alves M, et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2021;80(2):219-227.