Background Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is one of the leading cause of morbi-moratlity in SSc. Progression of ILD is variable among patients and some prognosis factors of mortality and progression have been suggested such as the extension of ILD on HRCT or the cutaneous extension of the disease. However, to date, there is no available study which assessed the prognostic information provided both by HRCT/pulmonary function tests (PFT) at baseline and clinical/biological characteristics of SSc.
Objectives To assess the prognosis value of ILD and SSc baseline date on the survival and progression-free survival.
Methods We restrospectively collected data of 75 consecutive SSc patients with ILD on HRCT and with a regular follow up with PFT. Progression of ILD was defined by a decrease of FVC≥10% and/or DLCO≥15% or the introduction of an immunosuppressive treatment. Each baseline HRCT was reviewed by 2 independent radiologists in order to assess the extension of disease and the proportion of ground-glass opacities and reticular pattern.
Results Overall survival was 97, 94, 90 and 85% at 1, 3, 5 and 10 years respectively. Significant prognosis factors were FVC, DLCO, age at ILD diagnosis, left ventricular ejection fraction, CRP, hemoglobin level and presence of anticentromere antibodies. Conversely, neither the subtypes of SSc, nor the sex, 6 minute walk test or the extension of ILD on the HRCT were significant prognosis factors for survival. DLCO was the independent prognosis factor of survival. Progression-free survival was 78, 50, 43 and 24% at 1, 3, 5 and 10 years respectively. Significant prognosis factors of progression-free survival were only NYHA functional class and the extension of ILD on the baseline HRCT whatever the proportion of ground-glass opacities or reticular pattern
Conclusions This study shows a good long term survival of patients with SSc-ILD in our center. However, the progression-free survival was much worse. The two prognosis factors of progression-free survival were the NYHA functional class and the extension of ILD on the baseline HRCT. Neither the subtype of SSc nor the subtype of autoantibodies were prognosis factor for progression-free survival.
Disclosure of Interest None Declared
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