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Total lung capacity is predictive of disease severity and survival in systemic sclerosis: A longitudinal analysis in 2347 patients from the French National Cohort Study.

Title: Total lung capacity is predictive of disease severity and survival in systemic sclerosis: A longitudinal analysis in 2347 patients from the French National Cohort Study.
Authors: Chaigne, Benjamin; Bense, A.; Aubourg, F.; Agard, C.; Allanore, Y.; Berezne, A.; Pugnet, G.; Hachulla, Eric; Cottin, V.; Hot, A.; Dunogue, B.; Kanagaratnam, A.; Palat, S.; Lescoat, A.; Berthier, S.; Chatelus, E.; Rivière, S.; Launay, David; Truchetet, M. E.; Dinh-Xuan, A. T.; Mouthon, L.
Contributors: Hôpital Cochin AP-HP; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP); Institute for Translational Research in Inflammation - U 1286 (INFINITE); Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire CHU Lille (CHRU Lille); Infections Virales et Pathologie Comparée - UMR 754 (IVPC); École Pratique des Hautes Études (EPHE); Université Paris Sciences et Lettres (PSL)-Université Paris Sciences et Lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL); Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE); Lille Inflammation Research International Center - U 995 (LIRIC); Institut Pasteur de Lille; Pasteur Network (Réseau International des Instituts Pasteur)-Pasteur Network (Réseau International des Instituts Pasteur)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire CHU Lille (CHRU Lille)
Source: ISSN: 0896-8411.
Publisher Information: CCSD; Elsevier
Publication Year: 2025
Collection: Université de Lyon: HAL
Subject Terms: Systemic sclerosis; Total lung capacity; Disease severity; Survival; [SDV]Life Sciences [q-bio]
Description: International audience ; BackgroundTotal lung capacity (TLC) is seldom assessed in the prediction of systemic sclerosis (SSc) disease severity.ObjectiveTo describe and analyse TLC in SSc.MethodsWe performed a retrospective multicentre study of SSc patients enrolled in the French national SSc cohort with at least one TLC assessment, described patients based on baseline TLC measurements, modelized TLC trajectories in SSc, and associated TLC measures with disease prognosis.ResultsTwo thousand three hundred and forty-seven patients were included in the study. Baseline TLC was associated with disease severity and survival, as well as with the occurrence of interstitial lung disease (ILD), lung fibrosis (LF), and pulmonary arterial hypertension (PAH). Individual TLC trajectories varied among patients. Different models of TLC trajectories were assessed using latent process mixed models. The best model showed that the vast majority of SSc patients had stable TLC trajectories and clustered patients into three groups predictive of SSc survival, ILD, LF, and PAH. Lastly, a 10 % decrease of TLC was found to be predictive of a 5 % decrease in forced vital capacity (FVC), a 10 % decrease in DLCO, and consequently an earlier predictive marker of ILD and LF than FVC.LimitationsRetrospective study.ConclusionTLC is predictive of disease severity and survival in SSc and SSc-ILD. This work suggests TLC as an earlier risk factor for ILD and LF than FVC in SSc.
Document Type: article in journal/newspaper
Language: English
Relation: info:eu-repo/semantics/altIdentifier/pmid/40037002; PUBMED: 40037002; WOS: 001442057100001
DOI: 10.1016/j.jaut.2025.103391
Availability: https://lilloa.hal.science/hal-05005965; https://lilloa.hal.science/hal-05005965v1/document; https://lilloa.hal.science/hal-05005965v1/file/1-s2.0-S0896841125000368-main.pdf; https://doi.org/10.1016/j.jaut.2025.103391
Rights: https://creativecommons.org/licenses/by/4.0/ ; info:eu-repo/semantics/OpenAccess
Accession Number: edsbas.14DEEB1E
Database: BASE