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Updated: Jun 18, 2026

04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Geoepidemiology of systemic sclerosis
1Université Paris Descartes, Service de Médecine Interne, hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux de Paris (AP-HP), Paris, France.
Autoimmunity Reviews
|November 13, 2009
Summary
Systemic sclerosis (SSc) is a rare fibrotic disease. Incidence and survival have improved due to increased awareness and better treatments, though pulmonary complications remain critical.
Area of Science:
- Rheumatology
- Epidemiology
- Internal Medicine
Background:
- Systemic sclerosis (SSc) is a rare, severe connective-tissue disease marked by skin fibrosis and organ involvement.
- Its rarity and varied presentation complicate epidemiological research.
- Prevalence varies globally, with higher rates in North America and Australia.
Purpose of the Study:
- To review the epidemiology of Systemic Sclerosis.
- To identify risk factors and survival trends in SSc patients.
Main Methods:
- Review of existing epidemiological studies on SSc.
- Analysis of prevalence, incidence, risk factors, and survival data.
- Examination of mortality causes and risk factors.
Main Results:
- SSc prevalence is 3-24 per 100,000; incidence increased from the 1950s-1980s, likely due to awareness.
- Risk factors include female sex, African origin, and exposure to silica and solvents.
- Ten-year survival improved from 50% to over 70%, with pulmonary fibrosis and hypertension as leading causes of death.
Conclusions:
- SSc epidemiology is influenced by geographic location, demographics, and environmental exposures.
- Improved survival highlights treatment advances, but pulmonary complications remain a significant challenge.
- Diffuse cutaneous forms and specific organ involvement are linked to mortality risk.
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