• Media type: E-Article
  • Title: Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region
  • Contributor: Lescoat, Alain; Huscher, Dörte; Schoof, Nils; Airò, Paolo; de Vries-Bouwstra, Jeska; Riemekasten, Gabriela; Hachulla, Eric; Doria, Andrea; Rosato, Edoardo; Hunzelmann, Nicolas; Montecucco, Carlomaurizio; Gabrielli, Armando; Hoffmann-Vold, Anna-Maria; Distler, Oliver; Ben Shimol, Jennifer; Cutolo, Maurizio; Allanore, Yannick; Cerinic, Marco Matucci; Walker, Ulrich; Iannone, Florenzo; Becvar, Radim; Bielecka, Otylia Kowal; Pizzorni, Carmen; Ciccia, Francesco; [...]
  • imprint: Oxford University Press (OUP), 2023
  • Published in: Rheumatology
  • Language: English
  • DOI: 10.1093/rheumatology/keac576
  • ISSN: 1462-0324; 1462-0332
  • Keywords: Pharmacology (medical) ; Rheumatology
  • Origination:
  • Footnote:
  • Description: <jats:title>Abstract</jats:title> <jats:sec> <jats:title>Objectives</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Material and methods</jats:title> <jats:p>Patients were clustered into seven geographical regions. Clinical characteristics and survival of patients with SSc-ILD were compared among these pre-determined regions.</jats:p> </jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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 &amp;lt;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).</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>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.</jats:p> </jats:sec>