| Title | Claps | Level | Year | L/Y |
|---|---|---|---|---|
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Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort.
13 auth. A. Hoffmann-Vold, H. Fretheim, A. Halse, M. Seip, H. Bitter, M. Wallenius, T. Garen, Anne Salberg, C. Brunborg, Ø. Midtvedt, ...
RATIONALE
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.
OBJECTIVE
Ev…
RATIONALE
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.
OBJECTIVE
Evaluate impact of ILD in a unique, nationwide, population based SSc cohort.
METHODS
ILD was assessed prospectively in the Norwegian SSc cohort (Nor-SSc) including all 815 SSc patients resident in the country in 2000-2012. Lung HRCTs were available for fibrosis quantification at baseline (n=650, 80%) and follow-up. Pulmonary function tests were assessed at baseline (n=703, 86%) and follow-up. Vital status and standardized mortality rates (SMR) were estimated at study end (2018) in the 630 incident Nor-SSc cases and 15 individually matched controls. Cumulative survival rates were computed.
MEASUREMENTS/MAIN RESULTS
At baseline, 50% of the SSc patients (n=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 >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-years survival rates correlated with presence or absence of lung fibrosis, being 83% and 80% with no fibrosis; and 69% and 56% with lung fibrosis (p=0.03).
CONCLUSION
The mere presence of ILD at baseline appears to affect outcome in SSc, suggesting that all SSc patients should undergo baseline PFT and lung HRCT screening to diagnose ILD early and tailor further management.
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26
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7 | 2019 |
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