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Progressive interstitial lung disease in patients with systemic sclerosis- associated interstitial lung disease in the EUSTAR database

Annals of Rheumatic Diseases Dec 03, 2020

Hoffmann-Vold AM, Allanore Y, Alves M, et al. - Among patients with systemic sclerosis-associated interstitial lung disease (ILD) (SSc-ILD), researchers investigated overall disease course, progression patterns as well as risk factors predictive for progressive ILD, utilizing data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up. This analysis involved 826 patients with SSc-ILD. Progressive ILD was experienced by 219 (27%) during 12 ± 3 months. Progressive ILD was detected in 23% to 27% of SSc-ILD patients, in each 12-month span, however, only a minority of patients exhibited progression in consecutive periods. Male gender, higher modified Rodnan skin score and reflux/dysphagia symptoms were identified as the strongest predictive factors for forced vital capacity (FVC) reduction over 5 years. Experts found that a heterogeneous and variable disease course was shown by SSc-ILD and therefore close monitoring is essential in all patients. New treatment concepts, with treatment initiation prior to the occurrence of FVC decline, should aim for prevention of progression to avert irreversible organ damage.

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