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Systemic sclerosis (SSc) is a rare connective tissue disease characterized by limited or diffuse skin hardening and thickening, and can be characterized by damage to internal organs such as the lungs, heart, kidneys, digestive tract, the lungs are characterized by interstitial pulmonary disease (ILD), ILD is the main cause of death in SSc patients, SSc-ILD prognosis, high mortality rate.
study used data from the European Steyring and Research (EUSTAR) database to determine the overall course, progression patterns and risk factors for progressive interstitiac pulmonary disease (ILD) in SSc-ILD patients.
qualified SSc-ILD patients were registered in the EUSTAR database and forced lung capacity (FVC± was measured after baseline and 12±3 months.
long-term progressive ILD and progression patterns were evaluated in patients who had multiple measurements of FVC.
potential predictive factors for ILD progress using multi-factor hybrid effect models.
included a total of 826 SSc-ILD patients.
in 12±3 months, 219 patients (27%) showed a moderate decrease in FVC (a decrease of 5%-10%) or a significant decrease (a decrease of 10%).
FVC measurements were performed on an average of 5 years of follow-up period, 535 patients (65%) were tested.
23%-27% of SSc-ILD patients showed progressive ILD every 12-month cycle, but only a few patients showed continuous progress.
most patients with progressive ILD (58%) had a slow decline in lung function, with more stabilization/improvement time than decline, while only 8% of patients had a sustained rapid decline in FVC.
178 patients (33%) had no decline in FVC.
the strongest predictive factors for FVC decline in five years were men, high improved Rodnan skin scores and reflow/difficulty swallowing symptoms.
SSc-ILD is heterogeneic and has a variable course of disease, it is important to follow up closely with all patients.
concept of starting treatment before FVC declines can avoid irreversible organ damage.
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