Lung function and skin fibrosis changes as predictors of survival in SSc-associated interstitial lung disease: a EUSTAR study

作者：Vincent Sobanski, Jeska de Vries‐Bouwstra, Anna‐Maria Hoffmann‐Vold, Dörte Huscher, Margarida Alves, Marco Matucci‐Cerinic, Gabriela Riemekasten, Mengtao Li, László Czirják, Otylia Kowal‐Bielecka, Yannick Allanore, Nils Schoof, Oliver Distler, EUSTAR Collaborators, Silvia Bellando-Randone, Ulrich A. Walker, Florenzo Iannone, Britta Maurer, Radim Bečvář, Antonella Riccardi, Elise Siegert, Simona Rednic, Jérôme Avouac, Carlomaurizio Montecucco, Patricia E. Carreira, Cecília Varjú, Carlo Chizzolini, Andrea Doria, Bernard Coleiro, Armando Gabrielli, Dominique Farge Bancel, Paolo Airó, Alexandra Balbir‐Gurman, Alessandro Giollo, Christopher P. Denton, Nemanja Damjanov, Jörg Henes, Vera Ortiz-Santamaría, Stefan Heitmann, Bojana Stamenković, Carlo Selmi, Mohammed Tikly, Lidia P Ananieva, Ariane L. Herrick, Ulf Müller-Ladner, Merete Engelhart, É. Hachulla, Valeria Riccieri, Ruxandra Ionescu, Ana Maria Gheorghiu, Jörg H. W. Distler, Francesca Ingegnoli, Vanessa Smith, Francesco Paolo Cantatore, Susanne Ullman, Piotr Wiland, Marie Vanthuyne, Juan José Alegre Sancho, Kristine Herrmann, Ellen De Langhe, Branimir Anić, Maria Üprus, Şule Yavuz, Carolina de, Souza Müller, Svetlana Agachi, D'Alessandro Mathieu, Kamal Solanki, Douglas J. Veale, Esthela Loyo, Edoardo Rosato, Edoardo Rosato, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuța, Peter M. Villiger, Jacob M. van Laar, Nihal Fathi, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Francesco Del Galdo, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, I. Castellví, Doron Rimar, Maura Couto, François Spertini, Sarah Kahl, Vivien Hsu, Thierry Martin, Lorinda S Chung, Tim Schmeiser, Dominik Majewski, Vera Bernardino, Konstantinos Fourlakis, Elena Rezuş · 发表于：Lara D. Veeken · 年份：2025 · DOI：10.1093/rheumatology/keaf264 · 被引用次数：7 · 研究领域：Systemic Sclerosis and Related Diseases、Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis、Inflammatory Myopathies and Dermatomyositis

OBJECTIVES: This study assessed how changes in lung function, skin fibrosis and digital ulceration (DU) burden predict mortality in patients with SSc-associated interstitial lung disease (SSc-ILD), the leading cause of death in SSc. METHODS: Adult SSc-ILD patients from the European Scleroderma Trials and Research (EUSTAR) database enrolled since January 2009 with a date of diagnosis, a follow-up visit for change evaluation within 12 months plus a further visit or mortality information were eligible. Twelve-month changes in lung function (per cent predicted forced vital capacity [FVC%pred] and diffusing capacity of the lungs for carbon monoxide [DLCO%pred]), modified Rodnan skin score (mRSS) and change in DU burden were assessed for associations with survival, using multivariable Cox regression analyses adjusted for age, sex, smoking status and immunosuppressive therapy. RESULTS: Of 893 SSc-ILD patients included, 94 (10.5%) died over a mean follow-up of 39.0 ± 23.9 months. Absolute deterioration in FVC >10%pred within 12 months (n = 78/638 evaluable) was predictive for decreased survival (hazard ratio [HR] 3.81; 95% CI 1.67-8.66), as were composite measures combining (i) >10% FVC decline or mRSS worsening (HR 2.82; 95% CI 1.43-5.56) and (ii) FVC decline ≥10% or 5-9% with DLCO decline ≥15% (HR 3.42; 95% CI 1.68-7.00), but not changes in DLCO, mRSS or DU burden alone. CONCLUSIONS: Changes in lung function and skin fibrosis within 12 months should be considered when evaluating ri...