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

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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 , 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 , Tianlu Li , 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 , Marko Barešić , Miroslav Mayer , Maria Üprus , Kati Otsa , Şule Yavuz , Carolina de Souza Müller , Svetlana Agachi , D'Alessandro Mathieu , Alessandra Vacca , Kamal Solanki , Douglas J. Veale , Esthela Loyo , Carmen Tineo , Edoardo Rosato , Edoardo Rosato , Cristina Tănăseanu , Rosario Foti , Codrina Ancuța , Peter M. Villiger , Sabine Adler , Jacob M. van Laar , Nihal Fathi , Paloma García de la Peña Lefebvre , Jorge Juan González Martín , Jean Sibilia , Ira Litinsky , Francesco Del Galdo , Lesley Ann Saketkoo , Eduardo Kerzberg , Washington Bianchi , Breno Valdetaro Bianchi , I. Castellví , Doron Rimar , Maura Couto , François Spertini , Sarah Kahl , Vivien Hsu , Thierry Martin , Leland W.K. Chung , Tim Schmeiser ·

YOU? · · 2025 · Open Access · · DOI: https://doi.org/10.1093/rheumatology/keaf264

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 risk of mortality. The effect of pharmacological treatments aiming at stabilization of these variables should be evaluated prospectively in clinical trials.

Related Topics

Medicine

Interstitial Lung Disease

Concepts

Medicine Interstitial lung disease Lung Lung function Pulmonary fibrosis Disease Lung disease Pathology Lung fibrosis Pulmonary function testing Internal medicine

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Type: article
Language: en
Landing Page: https://doi.org/10.1093/rheumatology/keaf264
PDF: https://academic.oup.com/rheumatology/advance-article-pdf/doi/10.1093/rheumatology/keaf264/63421845/keaf264.pdf
OA Status: hybrid
Cited By: 1
References: 50
Related Works: 10
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DOI: https://doi.org/10.1093/rheumatology/keaf264

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

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Language: en

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Publication year: 2025

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Publication date: 2025-06-03

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Authors: 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, 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, Tianlu Li, 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, Marko Barešić, Miroslav Mayer, Maria Üprus, Kati Otsa, Şule Yavuz, Carolina de Souza Müller, Svetlana Agachi, D'Alessandro Mathieu, Alessandra Vacca, Kamal Solanki, Douglas J. Veale, Esthela Loyo, Carmen Tineo, Edoardo Rosato, Edoardo Rosato, Cristina Tănăseanu, Rosario Foti, Codrina Ancuța, Peter M. Villiger, Sabine Adler, Jacob M. van Laar, Nihal Fathi, Paloma García de la Peña Lefebvre, Jorge Juan González Martín, Jean Sibilia, Ira Litinsky, Francesco Del Galdo, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, Breno Valdetaro Bianchi, I. Castellví, Doron Rimar, Maura Couto, François Spertini, Sarah Kahl, Vivien Hsu, Thierry Martin, Leland W.K. Chung, Tim Schmeiser

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Concepts: Medicine, Interstitial lung disease, Lung, Lung function, Pulmonary fibrosis, Disease, Lung disease, Pathology, Lung fibrosis, Pulmonary function testing, Internal medicine

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Cited by: 1

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abstract_inverted_index.be	223, 242
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abstract_inverted_index.(i)	173
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abstract_inverted_index.but	201
abstract_inverted_index.for	59, 92, 108, 118, 158
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abstract_inverted_index.&gt;10%	174
abstract_inverted_index.Conclusions	211
abstract_inverted_index.Scleroderma	41
abstract_inverted_index.information	71
abstract_inverted_index.1.43–5.56)	184
abstract_inverted_index.Twelve-month	74
abstract_inverted_index.associations	109
abstract_inverted_index.interstitial	23
abstract_inverted_index.1.67–8.66),	167
abstract_inverted_index.1.68–7.00),	200
abstract_inverted_index.[DLCO%pred]),	95
abstract_inverted_index.deterioration	145
abstract_inverted_index.multivariable	113
abstract_inverted_index.prospectively	244
abstract_inverted_index.stabilization	237
abstract_inverted_index.SSc-associated	22
abstract_inverted_index.&gt;10%pred	148
abstract_inverted_index.pharmacological	233
abstract_inverted_index.immunosuppressive	124
cited_by_percentile_year.max	95
cited_by_percentile_year.min	91
countries_distinct_count	11
institutions_distinct_count	105
sustainable_development_goals[0].id	https://metadata.un.org/sdg/3
sustainable_development_goals[0].score	0.8799999952316284
sustainable_development_goals[0].display_name	Good health and well-being
citation_normalized_percentile.value	0.89381933
citation_normalized_percentile.is_in_top_1_percent	False
citation_normalized_percentile.is_in_top_10_percent	True