Global trends in systemic sclerosis-related mortality, 2001–2023: an epidemiological analysis using World Health Organization mortality data - Report - MDSpire
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Global trends in systemic sclerosis-related mortality, 2001–2023: an epidemiological analysis using World Health Organization mortality data
Global Mortality Trends in Systemic Sclerosis, 2001–2023: WHO Data Analysis
Overview
This epidemiological study analyzed 85,291 systemic sclerosis (SSc)-related deaths from 74 countries between 2001 and 2023 using WHO mortality data. Findings reveal increasing age-standardized mortality rates, predominance in women, and regional variations, highlighting the ongoing global burden of SSc.
Background
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by multiple organ involvement and high morbidity and mortality. It predominantly affects women and has a pooled global prevalence of approximately 18.87 per 100,000 persons. Despite advances in treatment, SSc remains the rheumatic disease with the highest mortality, yet global mortality trends have not been comprehensively reported until now. This study fills that gap by analyzing WHO mortality data to identify temporal and demographic patterns in SSc-related deaths worldwide.
Data Highlights
Parameter
Value
Total SSc deaths (2001–2023)
85,291
Countries analyzed (LOESS)
74
Countries analyzed (Joinpoint)
47
Female proportion of deaths
79.40%
Age-specific mortality rate peak (80–84 years)
13.55 per 1,000,000
Crude mortality rate (2001)
1.97 per 1,000,000 (95% CI: 1.71–2.23)
Crude mortality rate (2023)
2.34 per 1,000,000 (95% CI: 2.01–2.68)
Leading cause of SSc death subtype
Other forms of SSc (M34.8, 48.64%)
Second leading cause
Unspecified SSc (M34.9, 35.43%)
Key Findings
From 2001 to 2023, age-standardized SSc mortality rates increased globally from 1.97 to 2.34 deaths per 1,000,000 population.
Women accounted for nearly 80% of SSc-related deaths, consistent with known female predominance in SSc.
Mortality rates increased with age, peaking in the 80–84-year age group before declining slightly in those over 85 years.
The majority of SSc deaths were attributed to other forms of SSc (48.64%) and unspecified SSc (35.43%), indicating challenges in subtype classification.
Data quality criteria limited inclusion to countries with medium to high-quality vital registration systems, ensuring reliability of mortality trends.
Regional and sociodemographic stratifications were performed but detailed results were not provided in the excerpt.
Clinical Implications
Clinicians should recognize the increasing global mortality burden of systemic sclerosis, particularly among older adults and women. The predominance of deaths classified under non-specific or other SSc subtypes underscores the need for improved diagnostic precision and organ-specific management. Public health strategies should target high-risk populations identified by age and sex to optimize resource allocation and improve outcomes.
Conclusion
This first global epidemiological assessment of SSc mortality using WHO data reveals rising mortality rates over two decades, with significant demographic disparities. These findings emphasize the urgent need for enhanced clinical awareness, improved disease classification, and targeted interventions to reduce SSc-related mortality worldwide.
References
Volkmann et al. 2023 -- Worldwide Patterns in Mortality Associated with Systemic Sclerosis
