Impact of evolving treatment patterns on interstitial lung disease progression in systemic sclerosis using the EUSTAR database.

Campochiaro, C.; Truchetet, ME.; Vonk, M.; De Luca, G.; Cuomo, G.; Ananieva, LP.; Hachulla, E.; Smith, V.; Gheorghiu, AM.; Becvar, R.; Carreira, P.; Hunzelmann, N.; Furst, DE.; Ortiz-Santamaria, V.; Del Galdo, F.; Matucci-Cerinic, M.; Hoffmann-Vold, AM.

Impact of evolving treatment patterns on interstitial lung disease progression in systemic sclerosis using the EUSTAR database.

All Authors

Campochiaro, C.

Truchetet, ME.

Vonk, M.

De Luca, G.

Cuomo, G.

Ananieva, LP.

Hachulla, E.

Smith, V.

Gheorghiu, AM.

Becvar, R.

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LTHT Author

Del Galdo, Francesco

LTHT Department

NIHR Leeds Biomedical Research Centre
Rheumatology

Publication Date

2026

Item Type

Journal Article

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. Copyright This article is protected by copyright. All rights reserved.