Global trends in systemic sclerosis-related mortality, 2001-2023: an epidemiological analysis using World Health...

This study aimed to evaluate the global trends in systemic sclerosis (SSc)-related mortality by age, sex, and geographic region. SSc is a multisystem autoimmune disease characterized by tissue fibrosis, vascular dysfunction, and multi-organ involvement, which is associated with a high mortality risk.

Using the World Health Organization Mortality Database, we examined trends in SSc-related crude mortality rates (SSc-CRs) and age-standardized mortality rates (SSc-ASMR) per 1,000,000 population from 2001 to 2023. Locally weighted regression was applied to visualize long-term patterns, and Joinpoint regression was used to assess the national trends from 2010 to 2023.

Across 74 countries, 85,291 SSc-related deaths were reported, with 79.41% occurring in females. The SSc-CR steadily increased from 1.97 (95% confidence interval [CI]: 1.71-2.23) in 2001 to 2.34 (95% CI: 2.01-2.68) in 2023, while the SSc-ASMR decreased from 1.58 (95% CI: 1.42-1.74) to 1.29 (95% CI: 1.08-1.50), respectively. Regionally, mortality was the highest in the Western Pacific region and declined in the Americas and Europe, with temporal fluctuations. The SSc-ASMR was highest in countries with a middle sociodemographic index (SDI).

While overall age-standardized mortality from SSc has declined in many regions, disparities persist. These results underscore the importance of sustaining research and enhancing disease awareness, as well as developing strategies to reduce mortality in high-risk populations and regions. Key Points • First global analysis of mortality trends across 74 countries (2001-2023) • Age-standardized mortality declined globally, but crude mortality increased, with persistent female predominance • Findings highlight need for targeted strategies, early diagnosis, and improved care to reduce mortality.