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Hoffmann-Vold, Anna-Maria; Fretheim, Håvard; Halse, Anne-Kristine; Seip, Marit; Bitter, Helle; Wallenius, Marianne; Garen, Torhild; Salberg, Anne; Brunborg, Cathrine; Midtvedt, Øyvind; Lund, May Brit; Aaløkken, Trond M; Molberg, Øyvind
American journal of respiratory and critical care medicine, 11/2019, Volume: 200, Issue: 10Journal Article
Interstitial lung disease (ILD) represents a major challenge in systemic sclerosis (SSc), but there are no precise, population-based data on its overall impact, limiting opportunities for screening and management strategies. Evaluate impact of ILD in a unique, nationwide, population-based SSc cohort. ILD was assessed prospectively in the Norwegian SSc (Nor-SSc) cohort, including all 815 patients with SSc resident in the country from 2000 to 2012. Lung high-resolution computed tomography (HRCT) scans were available for fibrosis quantification at baseline ( = 650, 80%) and follow-up. Pulmonary function tests were assessed at baseline ( = 703, 86%) and follow-up. Vital status and standardized mortality ratios (SMRs) were estimated at study end (2018) in the 630 incident Nor-SSc cases and 15 individually matched control subjects. Cumulative survival rates were computed. At baseline, 50% of the subjects with SSc ( = 324) had ILD by HRCT and 46% displayed pulmonary function declines consistent with ILD progression. Mortality correlated with extent of lung fibrosis as SMR increased from 2.2 with no fibrosis to 8.0 with greater than 25% fibrosis. SMR was inversely related to baseline FVC% and increased at all FVC levels below 100%. In patients with normal-range baseline FVC (80-100%), the 5- and 10-year survival rates correlated with presence or absence of lung fibrosis, being 83% and 80%, respectively, with no fibrosis and 69% and 56%, respectively, with lung fibrosis ( = 0.03). The mere presence of ILD at baseline appears to affect outcome in SSc, suggesting that all patients with SSc should undergo a baseline pulmonary function test and lung HRCT screening to diagnose ILD early and tailor further management.
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