The incidence of interstitial lung disease in patients with systemic sclerosis: rate, risk factors and prognostic implications in a EUSTAR cohort analysis (CP 133)

Petelytska L, Tofani L, Velauthapillai A, Dobrota R, Becker MO, Mihai C, Muraru S, Elhai M, Jordan S, Hachulla E, Müller-Ladner U, Siegert E, Allanore Y, Riemekasten G, Bergmann C, Becvar R, Solanki K, Anic B, Szabo I, Stamenkovic B, Distler J, Carreira PE, Cuomo G, Castellví I, Balbir-Gurman A, Airò P, Litinsky I, Saketkoo LA, Bertaccini B, Vonk MC, de Vries-Bouwstra J, Hoffmann-Vold AM, Matucci-Cerinic M, Distler O, Bruni C (2026)

Publication Type: Journal article

Publication year: 2026

Journal

Book Volume: 85

Pages Range: 669-679

Journal Issue: 4

DOI: 10.1016/j.ard.2025.12.008

Abstract

Objectives: Interstitial lung disease (ILD) carries significant morbidity and mortality risk in systemic sclerosis (SSc). We aimed to estimate the incidence of new-onset SSc-ILD and the associated risk factors, as well as its impact on the prognosis. Methods: Patients classified as having SSc, with the absence of ILD signs on high-resolution computed tomography (HRCT) at baseline and having at least 1 follow-up visit with available HRCT data, were selected. SSc-ILD incidence was calculated as a rate per 100 person-years. Predictors of new-onset ILD and risk factors for ILD progression and mortality were chosen according to the literature and expert opinion. Risk factors for new-onset ILD, as well as its prognostic impact on ILD progression and mortality, were tested by generalised logistic estimating equation and Cox regression models, respectively. Results: Among 5331 patients with SSc with negative baseline HRCT, the incidence of new-onset ILD was 3.83 cases per 100 person-years. Notably, there was a continuous detection of new ILD onset up to 10 years from baseline. Risk factors for new-onset ILD included New York Heart Association stage ≥2, muscle weakness, high inflammatory markers, and SSc-specific autoantibodies, but not disease duration. Despite a lower risk of ILD progression compared with prevalent ILD diagnosed at baseline, incident ILD still carried an increased risk for mortality, which was almost double when compared with ILD-negative cases. Conclusions: Patients with SSc should be considered for regular screening following a negative baseline HRCT, in particular when carrying high-risk features for new ILD onset, given its incidence and prognostic implications.

Authors with CRIS profile

Involved external institutions

Charité - Universitätsmedizin Berlin Germany (DE) Hôpital Cochin France (FR) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) Te Whatu Ora Health New Zealand New Zealand (NZ) Zagreb University Hospital Centre / Klinički bolnički centar Zagreb (KBC) Croatia (HR) Iuliu Hațieganu University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie "Iuliu Hațieganu" (UMF Cluj) Romania (RO) Universitätsklinikum Düsseldorf Germany (DE) Hospital Universitario 12 de Octubre Spain (ES) Universitätsspital Zürich (USZ) Switzerland (CH) Università degli Studi di Firenze / University of Florence Italy (IT) Institute for Translational Research in Inflammation (INFINITE) France (FR) Justus-Liebig-Universität Gießen Germany (DE) Università degli studi della Campania Luigi Vanvitelli Italy (IT) Hospital de la Santa Creu i Sant Pau Spain (ES) Rambam Health Care Campus Israel (IL) Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia Italy (IT) Tel Aviv Sourasky Medical Center / Ichilov Hospital Israel (IL) Tulane University United States (USA) (US) Radboud University Nijmegen Medical Centre / Radboudumc of voluit Radboud Universitair Medisch Centrum (UMC) Netherlands (NL) Leiden University Medical Center Netherlands (NL) Ospedale San Raffaele (früher: Centro San Raffaele del Monte Tabor Foundation) Italy (IT)

How to cite

APA:

Petelytska, L., Tofani, L., Velauthapillai, A., Dobrota, R., Becker, M.O., Mihai, C.,... Bruni, C. (2026). The incidence of interstitial lung disease in patients with systemic sclerosis: rate, risk factors and prognostic implications in a EUSTAR cohort analysis (CP 133). Annals of the Rheumatic Diseases, 85(4), 669-679. https://doi.org/10.1016/j.ard.2025.12.008

MLA:

Petelytska, Liubov, et al. "The incidence of interstitial lung disease in patients with systemic sclerosis: rate, risk factors and prognostic implications in a EUSTAR cohort analysis (CP 133)." Annals of the Rheumatic Diseases 85.4 (2026): 669-679.

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