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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 Oct 17, 2020

Hoffmann-Vold AM, Allanore Y, Alves M, et al. - This study utilizing data from the European Scleroderma Trials And Research database with long-term follow-up was conducted to determine overall disease course, progression patterns as well as risk factors predictive for progressive interstitial lung disease (ILD) among patients suffering from systemic sclerosis-associated ILD (SSc-ILD). Overall 826 patients were analyzed. Progressive ILD was detected in 219 (27%) during 12 ± 3 months. In each 12-month period, progressive ILD was seen in 23% to 27% of SSc-ILD patients, but only a minority of cases demonstrated 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. Overall, findings revealed a heterogeneous and variable disease course of SSc-ILD, and therefore it is essential to perform close monitoring in all patients. New treatment concepts, with treatment start prior to FVC reduction happens, should aim for prophylaxis of progression to avert irreversible organ damage.

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