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Related Experiment Video

Updated: Aug 26, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region.

Alain Lescoat1,2, Dörte Huscher3, Nils Schoof4

  • 1Department of Internal Medicine and Clinical Immunology, CHU Rennes, University of Rennes 1, Rennes, France.

Rheumatology (Oxford, England)
|October 12, 2022
PubMed
Summary

Geographical region significantly impacts the prevalence, presentation, and treatment of Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD). Management and survival rates for SSc-ILD vary globally, indicating a need for standardized care.

Keywords:
SScautoantibodiesinterstitial lung diseaselung fibrosisscleroderma

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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.