Lescoat, A., Huscher, D., Schoof, N. et al. (204 more authors) (2023) Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region. Rheumatology, 62 (6). pp. 2178-2188. ISSN 1462-0324
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.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Keywords: | interstitial lung disease, lung fibrosis, scleroderma, SSc, autoantibodies |
Dates: |
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Institution: | The University of Leeds |
Academic Units: | The University of Leeds > Faculty of Medicine and Health (Leeds) > School of Medicine (Leeds) |
Depositing User: | Symplectic Publications |
Date Deposited: | 07 Apr 2025 10:42 |
Last Modified: | 08 Apr 2025 14:54 |
Published Version: | https://academic.oup.com/rheumatology/article/62/6... |
Status: | Published |
Publisher: | Oxford University Press |
Identification Number: | 10.1093/rheumatology/keac576 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:225211 |