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

Alain Lescoat*, Dörte Huscher, Nils Schoof, Paolo Airò, Jeska De Vries-Bouwstra, Gabriela Riemekasten, Eric Hachulla, Andrea Doria, Edoardo Rosato, Nicolas Hunzelmann, Carlomaurizio Montecucco, Armando Gabrielli, Anna Maria Hoffmann-Vold, Oliver Distler, Jennifer Ben Shimol, Maurizio Cutolo, Yannick Allanore

*Corresponding author for this work
3 Citations (Scopus)


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.

Original languageEnglish
JournalRheumatology (United Kingdom)
Issue number6
Pages (from-to)2178-2188
Number of pages11
Publication statusPublished - 01.06.2023

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)

DFG Research Classification Scheme

  • 205-18 Rheumatology
  • 204-05 Immunology


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