Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis

Abstract

Objectives We aimed to conduct a systematic review and meta-analysis on the incidence and prevalence of SSc covering the entire literature. Methods This study followed the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Statement of 2009. We conducted a systematic search in MEDLINE, Web Of Science, and EMBASE to identify articles reporting incidence and/or prevalence of SSc. Two authors conducted the search, reviewed articles for inclusion and extracted relevant data. We used random-effects models to estimate the pooled prevalence and incidence of SSc and performed subgroup analyses by sex, case definition, and region to investigate heterogeneity. We explored the association between calendar period and reported estimates using meta-regression. Results Among 6983 unique records identified, we included 61 studies of prevalence and 39 studies of incidence in the systematic review. The overall pooled prevalence of SSc was 17.6 (95% CI 15.1-20.5) per 100000 and the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1-1.9) per 100000 person-years. We observed significant regional variations in reported estimates; studies conducted in North America reported considerably higher estimates than other regions. The pooled incidence and prevalence in women were 5 times higher than in men. More recent studies reported higher estimates than older ones. Conclusion In this comprehensive review of the incidence and prevalence of SSc across the world, there was large heterogeneity among estimates, which should be taken into consideration when interpreting the results.

Systemic sclerosis in Asians: Are there racial differences?

“…A recent meta-analysis showed that the overall pooled prevalence of SSc globally was 17.6 per 100,000, and the overall pooled incidence rate of SSc was 1.4 per 100,000 person-years. 15 SSc prevalence is higher in the United States and Australia (25 and 24 per 100,000, respectively) than in Europe (15 per 100,000) and Asia (6.8 per 100,000). 15 The incidence rate of SSc is also higher in the United States compared to Asia (1.5-2.0 vs 0.9 per 100,000 person-years).…”

A Narrative Review of Pathogenetic and Histopathologic Aspects, Epidemiology, Classification Systems, and Disease Outcome Measures in Systemic Sclerosis

“…A first explanation of this observed variability could be the existence of significant differences in the design and methods in different studies, concerning both case identification (data sources and case ascertainment) and definition [76][77][78][79]. In particular, although sometimes used also for prevalence and incidence evaluation, 1980 classification criteria suffered from poor sensitivity for early SSc and lcSSc, and were therefore not suitable to this task, inducing other groups [80,81] to use also the classification criteria early SSc proposed by LeRoy and Medsger in 2001 [47].…”

“…Geographical Variability A third reason for the variability in the observed incidence and prevalence could be related to geographical differences in SSc distribution that might also contribute to the variation of demographic parameters. Information on this issue from some large areas of the world (e.g., Africa and South America) is scanty [77]. The disease is more common in North America or Australia than in East Asia [77,79].…”

Systemic sclerosis‐associated interstitial lung disease in a Vietnamese adult female: Case report and literature review

“…Systemic sclerosis (SSc) is characterized by microvascular damage and generalized fibrosis in the skin and visceral organs. 1 , 2 , 3 This is a rare disease, with an estimated global prevalence of 3–24 per 100,000. 4 The pooled prevalence estimate in Asia is 6.8 per 100,000.…”

“…4 The pooled prevalence estimate in Asia is 6.8 per 100,000. 3 Lung fibrosis occurs in approximately 80% of patients with SSc; 25%-30% develop progressive interstitial lung disease (ILD), 2 which is a leading cause of morbidity and mortality, with a 10-year mortality of up to 40%. 1 Early diagnosis of SSc-ILD is crucial to initiate treatment and to prevent disease progression.…”