Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region.

Lescoat A, Huscher D, Schoof N, Airo P, De Vries-Bouwstra J, Riemekasten G, Hachulla E, Doria A, Rosato E, Hunzelmann N, Montecucco C, Gabrielli A, Hoffmann-Vold AM, Distler O, Ben Shimol J, Cutolo M, Allanore Y (2023)

Publication Type: Journal article

Publication year: 2023

Journal

Book Volume: 62

Pages Range: 2178-2188

Journal Issue: 6

DOI: 10.1093/rheumatology/keac576

Abstract

OBJECTIVES: The prevalence and characteristics of SSc-associated interstitial lung disease (SSc-ILD) vary between geographical regions worldwide. The objectives of this study were to explore the differences in terms of prevalence, phenotype, treatment and prognosis in patients with SSc-ILD from predetermined geographical regions in the EUSTAR database. MATERIAL AND METHODS: Patients were clustered into seven geographical regions. Clinical characteristics and survival of patients with SSc-ILD were compared among these pre-determined regions. RESULTS: For baseline analyses, 9260 SSc patients were included, with 6732 for survival analyses. The prevalence of SSc-ILD in the overall population was 50.2%, ranging from 44.0% in 'Western Europe and Nordic countries' to 67.5% in 'Eastern European, Russia and Baltic countries'. In all regions, anti-topoisomerase antibodies were associated with SSc-ILD. Management also significantly differed; mycophenolate mofetil was prescribed at baseline in 31.6% of patients with SSc-ILD in 'America (North and South)' and 31.7% in 'Middle East' but only 4.3% in 'Asia and Oceania' (P <0.0001). Patients from 'America (North and South)' and 'Middle East' had the highest survival rate at the end of follow-up (85.8% and 85.2%, respectively). CONCLUSIONS: Our study highlights key differences among regions in terms of clinical presentation and prognosis of SSc-ILD. This work also demonstrates that the management of SSc-ILD is highly variable among the different regions considered, suggesting that efforts are still needed for the standardization of medical practice in the treatment of this disease.

Involved external institutions

Université de Rennes 1 / University of Rennes 1 France (FR) Charité - Universitätsmedizin Berlin Germany (DE) Boehringer Ingelheim Pharma GmbH & Co. KG Germany (DE) Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia Italy (IT) Leiden University Netherlands (NL) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) LILLE 1 University - Science and Technology France (FR) University of Padua / Universita degli Studi di Padova Italy (IT) Università degli studi "La Sapienza" Italy (IT) Universität zu Köln Germany (DE) Università degli Studi di Pavia Italy (IT) Università Politecnica delle Marche (UNIVPM) / Marche Polytechnic University Italy (IT) Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet Norway (NO) Universitätsspital Zürich (USZ) Switzerland (CH) Wolfson Medical Center Israel (IL) University of Genova / Università degli Studi di Genova Italy (IT) University of Paris 5 - René Descartes / Université Paris V René Descartes France (FR)

How to cite

APA:

Lescoat, A., Huscher, D., Schoof, N., Airo, P., De Vries-Bouwstra, J., Riemekasten, G.,... Allanore, Y. (2023). Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region. Rheumatology, 62(6), 2178-2188. https://dx.doi.org/10.1093/rheumatology/keac576

MLA:

Lescoat, Alain, et al. "Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region." Rheumatology 62.6 (2023): 2178-2188.

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