Abstract
Background: The treatment landscape for systemic sclerosis-associated interstitial lung disease (SSc-ILD) has evolved with increasing immunosuppressive (IST) and anti-fibrotic therapies available. However, their real-world use remains unclear.
Objectives: To analyze treatment trends and the effect of IST and anti-fibrotic therapies on ILD progression using the EUSTAR database.
Methods: We included SSc-ILD patients meeting the 2013 ACR/EULAR criteria with high-resolution CT-confirmed ILD, pulmonary function, and therapy data, grouped into four time periods (≤2006, 2007-2011, 2012-2016, ≥2017). We analyzed IST initiation, switching, discontinuation, and combination therapy. ILD progression was defined as a decline in %FVC ≥5% or %DLCO ≥10% over 12 ± 3 months.
Results: Among 1,409 patients, IST use at first evaluation increased significantly from 13.6% (≤2006) to 57.4% (≥2017, p<0.001). Mycophenolate mofetil emerged as the most prescribed IST (7% to 57%, p<0.001). Combination therapy rose from 17.9% to 26.9% (p<0.001), while ILD progression rates declined from 21.3% (2007-2011) to 12.1% (≥2017, p<0.001). In the ≥2017 cohort, logistic regression showed shorter disease duration (Odds ratio (OR) 0.991, 95%CI 0.987-0.996, p <0.001) and myositis (OR 9.9, 95% CI 1.94-51.76, p=0.006) were associated with therapy initiation, while switching was higher in patients with a higher mRSS (OR 1.03, 95%CI 1.00-1.06, p=0.035) and in patients with arthritis (OR 3.03, 95% CI 1.55-5.94, p=0.001). Lastly, combination therapy was associated with younger age, higher dyspnea class and arthritis.
Conclusions: Our findings reveal a significant evolution in clinical practice. However, continued disease progression emphasizes the need for more effective therapeutic approaches.
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