Impact of Evolving Treatment Patterns on Interstitial Lung Disease Progression in Systemic Sclerosis Using the European Scleroderma Trials and Research Database.

Authors

Corrado Campochiaro, Marie-Elise Truchetet, Madelon Vonk, Giacomo De Luca, Giovanna Cuomo, Lidia P Ananieva, Eric Hachulla, Vanessa Smith, Ana Maria Gheorghiu, Radim Becvar, Patricia Carreira, Nicolas Hunzelmann, Daniel E Furst, Vera Ortiz-Santamaria, Francesco Del Galdo, Marco Matucci-Cerinic, Anna-Maria Hoffmann-Vold, EUSTAR collaborators

Published in

Arthritis & rheumatology (Hoboken, N.J.). Volume 78. Issue 8. Pages 1755-1764. Epub Mar 29, 2026.

Abstract

The treatment landscape for systemic sclerosis-associated interstitial lung disease (SSc-ILD) has evolved with increasingly available immunosuppressive therapies (ISTs) and antifibrotic treatments. However, their real-world use remains unclear. The objective of this study was to analyze treatment trends and the effect of IST and antifibrotic treatments on ILD progression using the European Scleroderma Trials and Research database.
We included patients with SSc-ILD meeting the 2013 American College of Rheumatology/EULAR criteria with high-resolution computed tomography-confirmed ILD, pulmonary function, and therapy data, grouped into four time periods (≤2006, 2007-2011, 2012-2016, and ≥2017). We analyzed IST initiation, switching, discontinuation, and combination therapy. ILD progression was defined as a decline in the percentage of predicted forced vital capacity of 5% or greater or the percentage of predicted diffusing capacity of the lungs for carbon monoxide of 10% or greater over 12 ± 3 months.
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), whereas ILD progression rates declined from 21.3% (2007-2011) to 12.1% (≥2017) (P < 0.001). In the 2017 and later cohort, logistic regression showed shorter disease duration (odds ratio [OR] 0.991, 95% confidence interval [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, whereas switching was higher in patients with a higher modified Rodnan skin score (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). Last, combination therapy was associated with younger age, higher dyspnea class, and arthritis.
Our findings reveal a significant evolution in clinical practice. However, continued disease progression emphasizes the need for more effective therapeutic approaches.

PMID:
41568546
Bibliographic data and abstract were imported from PubMed on 14 Sep 2026.

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