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

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

doi:10.1002/art.70043

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

Arthritis Rheumatol. 2026 Jan 22. doi: 10.1002/art.70043. Online ahead of print.

Authors

Corrado Campochiaro¹, Marie-Elise Truchetet², Madelon Vonk³, Giacomo De Luca¹, Giovanna Cuomo⁴, Lidia P Ananieva⁵, Eric Hachulla⁶, Vanessa Smith⁷, Ana Maria Gheorghiu⁸, Radim Becvar⁹, Patricia Carreira¹⁰, Nicolas Hunzelmann¹¹, Daniel E Furst¹², Vera Ortiz-Santamaria¹³, Francesco Del Galdo¹⁴, Marco Matucci-Cerinic¹, Anna-Maria Hoffmann-Vold^{15

16}; EUSTAR collaborators

Affiliations

¹ Unit of Immunology, Rheumatology, Allergy and Rare Diseases, Inflammation, Fibrosis and Ageing Initiative, Scientific Institute for Research Hospitalization and Healthcare San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy.
² Department of Rheumatology, University of Bordeaux, Bordeaux, France.
³ Department of Rheumatology, Radboud University Medical Center, Nijmegen, the Netherlands.
⁴ Department of Rheumatology, University of Naples Federico II, Italy.
⁵ V.A. Nasanova Institute of Rheumatology, Moscow, Russia.
⁶ Department of Internal Medicine and Clinical Immunology, Referral Centre for Rare Systemic Autoimmune Diseases North of France, North-West, Mediterranean and Guadeloupe, Centre Hospitalier Universitaire de Lille, University of Lille, French National Institute of Health and Medical Research, Lille, France.
⁷ Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
⁸ Department of Rheumatology, Bucharest University Emergency Hospital, Bucharest, Romania.
⁹ Department of Internal Medicine, Charles University, Prague, Czech Republic.
¹⁰ Department of Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain.
¹¹ Department of Dermatology, University Hospital of Cologne, Cologne, Germany.
¹² David Geffen School of Medicine, University of California Los Angeles.
¹³ Rheumatology Unit, Vall d'Hebron University Hospital, Barcelona, Spain.
¹⁴ Department of Rheumatology, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom.
¹⁵ Department of Rheumatology, Oslo University Hospital, Oslo, Norway.
¹⁶ Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.

PMID: 41568546
DOI: 10.1002/art.70043

Abstract

Objective: 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.

Methods: 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.

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), 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.

Conclusion: Our findings reveal a significant evolution in clinical practice. However, continued disease progression emphasizes the need for more effective therapeutic approaches.