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

Abstract

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.

Anti-U11/U12 Antibodies as a Rare but Important Biomarker in Patients with Systemic Sclerosis: A Narrative Review

“…During the last years, special focus was placed on lung disease in SSc patients. Consequently, biomarkers such as anti-U11/U12 antibodies might be useful to identify patients with rapidly progressing ILD [ 1 , 49 ], especially since the classification criteria marker anti-Scl-70, ACA and anti-RNA Pol III do not effectively allow for stratification, albeit still being useful f identify patients at risk for SSc-ILD [ 49 , 50 , 51 ]. Similarly, it must be determined to what extent these antibodies, in conjunction with other risk factors for SSc-ILD, such as the diffuse cutaneous subset or early disease onset, may identify patients that warrant close monitoring or early and aggressive intervention.…”

Health Equity in Rheumatology: A Global Health Perspective

“…5 The study also found significant differences in the clinical presentation and management of systemic sclerosisinduced interstitial lung disease across seven geographic regions in Europe, highlighting variations in management and the need to standardize evidence-based clinical guidelines. 6 DOI: https://doi.org/10.17925/RMD.2023. 2.1.42 touchREVIEWS in RMD Similarly, disparities are also rooted in the variable incidences and presentation of rheumatic disease in patients of different sexes and genders.…”