1. Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region.
- Author
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Lescoat, Alain, Huscher, Dörte, Schoof, Nils, Airò, Paolo, Vries-Bouwstra, Jeska de, Riemekasten, Gabriela, Hachulla, Eric, Doria, Andrea, Rosato, Edoardo, Hunzelmann, Nicolas, Montecucco, Carlomaurizio, Gabrielli, Armando, Hoffmann-Vold, Anna-Maria, Distler, Oliver, Shimol, Jennifer Ben, Cutolo, Maurizio, Allanore, Yannick, and collaborators, the EUSTAR
- Subjects
AUTOANTIBODIES ,SYSTEMIC scleroderma ,INTERSTITIAL lung diseases ,MYCOPHENOLIC acid ,RISK assessment ,SURVIVAL analysis (Biometry) ,DISEASE prevalence ,DESCRIPTIVE statistics ,RESEARCH funding ,PHENOTYPES ,DISEASE complications ,SYMPTOMS ,THERAPEUTICS - 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. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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