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Meta-Analysis
. 2021 Jul 1;60(7):3121-3133.
doi: 10.1093/rheumatology/keab190.

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

Majd Bairkdar  1 , Marios Rossides  1 , Helga Westerlind  1 , Roger Hesselstrand  2 , Elizabeth V Arkema  1 , Marie Holmqvist  1   3
Affiliations
Meta-Analysis

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

Majd Bairkdar et al. Rheumatology (Oxford). .

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 100 000 and the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1, 1.9) per 100 000 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 five 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.

Keywords: incidence; meta-analysis; prevalence; systematic review; systemic sclerosis.

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Figures

<sc>Fig</sc>. 1
Fig. 1
PRISMA 2009 flow diagram for process and outcome of the study selection strategy PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses.
<sc>Fig</sc>. 2
Fig. 2
Meta-analysis of SSc prevalence per 100 000, stratified by case definition method ICD: International Classification of Diseases.
<sc>Fig</sc>. 3
Fig. 3
Meta-analysis of SSc prevalence per 100 000, stratified by region
<sc>Fig</sc>. 4
Fig. 4
Meta-analysis of SSc incidence per 100 000 person-years, stratified by case definition method ICD: International Classification of Diseases.
<sc>Fig</sc>. 5
Fig. 5
Meta-analysis of SSc incidence per 100 000 person-years, stratified by region
See this image and copyright information in PMC

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