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Objectives The prevalence and characteristics of systemic sclerosis-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. 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 & Nordic countries” to 67.5% in “Eastern European, Russia & 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 & South)” and 31.7% in “Middle East” but only 4.3% in “Asia & Oceania” (P < 0.0001). Patients from “America (North & South)” and “Middle East” had the highest survival rate at the end of follow-up (85.8% and 85.2%, respectively). Conclusion 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 standardisation of medical practice in the treatment of this disease.

Objectives To estimate the long-term exposure to glucocorticoids (GC), the factors associated with, and the variations in prescribing practices over time and across recruiting countries. Methods We included patients with systemic sclerosis (SSc) having a visit in the EUSTAR database from January 2013 onward. We analyzed the prevalence and the main features of GC users, the exposure to GC over time, and the respective dosages. Multivariable linear regression analysis identified factors associated with GC intake duration. Time trends, and variations in GC utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations. Results The 9819 patients included were mostly females (85%), lcSSc (73%), median age 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs. 29% lcSSc) were on GC, median dose 7.5 mg/day. GC users were more frequently males, dcSSc, anti-Scl70 positive, with a more severe disease. On average, GC users spent 25% of their follow-up time (median 33.2 months) on GC, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed-up for >1 year, had received GC for >6 and >12 months, respectively. In multivariable analysis, patient and disease’s characteristics poorly explained the variability of GC exposure (adjusted-R2 = 0.06, P < 0.001). GC utilization varied within and across countries, and gradually decreased over time (36% in 2013 vs. 23% in 2018). Conclusions GC are widely and long-term prescribed in SSc, with significant between- and within-country(ies) differences. A gradual decrease in their utilization is observed.