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

Area of Science:

• Rheumatology
• Pulmonology
• Epidemiology

Background:

• Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD) exhibits geographical variations in prevalence and characteristics.
• Understanding these regional differences is crucial for effective SSc-ILD management.

Purpose of the Study:

• To investigate geographical disparities in SSc-ILD prevalence, phenotype, treatment, and prognosis.
• To analyze data from the EUSTAR database to identify regional variations.

Main Methods:

• Patients with SSc-ILD were categorized into seven geographical regions.
• Clinical characteristics and survival rates were compared across these regions.

Main Results:

• SSc-ILD prevalence ranged from 44.0% in Western Europe/Nordic countries to 67.5% in Eastern Europe/Russia/Baltic countries.
• Anti-topoisomerase antibodies were consistently associated with SSc-ILD across all regions.
• Treatment with mycophenolate mofetil varied significantly by region (e.g., 31.6% in Americas vs. 4.3% in Asia/Oceania).
• Highest survival rates were observed in patients from the Americas (85.8%) and the Middle East (85.2%).

Conclusions:

• Significant regional differences exist in SSc-ILD clinical presentation and prognosis.
• Treatment strategies for SSc-ILD are highly variable globally.
• Standardization of medical practice for SSc-ILD treatment is necessary.