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

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

Survival in Swedish patients with systemic sclerosis : A nationwide population-based matched cohort study

Objectives: To conduct the first-ever nationwide, population-based cohort study investigating survival patterns of all patients with incident SSc in Sweden compared with matched individuals from the Swedish general population. Methods: We used the National Patient Register to identify patients with incident SSc diagnosed between 2004 and 2015 and the Total Population Register to identify comparators (1:5), matched on sex, birth year and residential area. We followed them until death, emigration or the end of 2016. Follow-up of the general population comparators started the same date as their matched patients were included. We estimated all-cause survival using the Kaplan-Meier method, crude mortality rates and hazard ratios (HRs) using flexible parametric models. Results: We identified 1139 incident patients with SSc and 5613 matched comparators. The median follow-up was 5.0 years in patients with SSc and 6.0 years for their comparators. During follow-up, 268 deaths occurred in patients with SSc and 554 in their comparators. The 5-year survival was 79.8% and the 10-year survival was 67.7% among patients with SSc vs 92.9% and 84.8%, respectively, for the comparators (P < 0.0001). The mortality rate in patients with SSc was 42.1 per 1000 person-years and 15.8 per 1000 person-years in their comparators, corresponding to an HR of 3.7 (95% CI 2.9, 4.7) at the end of the first year of follow-up and 2.0 (95% CI 1.4, 2.8) at the end of the follow-up period. Conclusion: Despite advances in understanding the disease and in diagnostic methods over the past decades, survival is still severely impacted in Swedish patients diagnosed with SSc between 2004 and 2015

Incidence and prevalence of systemic sclerosis in Sweden, 2004–2015, a register-based study

Objectives: we aim to present an in-depth report of the incidence and prevalence of systemic sclerosis (SSc) in Sweden in a nation-wide register-based study covering the entire Swedish population Methods: each individual residing in Sweden is given a unique personal identity number. We linked the National Patient Register and the Total Population Register to identify 1) patients with prevalent SSc on 2015–12–31 and 2) patients with incident SSc during the time period 2004–2015 based on ICD-10 codes. We estimated prevalence and incidence overall and stratified on age, sex, and county. Results: we identified 1774 prevalent cases, median age was 65 years (IQR 19.2) and 84% were women. The point prevalence estimate was 22.7 per 100,000 (95%CI 13.3–32.0). 1139 individuals were newly diagnosed with SSc during 2004–2015 with a median age of 60 years (IQR 20.6) and 80% were women. The mean standardized incidence was 11.9 per 1,000,000 person-years (95%CI 5.1–18.7). The annual incidence remained stable over the study period. Women had five times higher incidence and prevalence than men. The highest prevalence stratified by age strata was observed in the group aged 70–79. Conclusion: SSc incidence and prevalence in Sweden are comparable to estimates from southern Europe, as opposed to the previous assumption of lower occurrence in northern Europe. We further observe that SSc incidence has been rather constant throughout recent years in Sweden with no obvious increase