The economic burden of systemic sclerosis-A systematic review.

AIM: Systemic sclerosis (SSc) is a rare, chronic autoimmune disease associated with a substantial economic burden. This study aimed to assess the costs associated with SSc and to identify major cost drivers. METHODS: A systematic search was conducted in MEDLINE and Embase to identify relevant studies. Two independent reviewers evaluated studies based on inclusion/exclusion criteria and performed data extraction. Costs were converted into 2017 US dollars by purchasing power parity. The review was conducted following the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guideline. RESULTS: The original literature search identified 113 potentially relevant citations, of which 10 articles met all the inclusion/exclusion criteria and were included in the data extraction and analysis. The identified studies evaluated costs associated with SSc in 11 countries from North America, Europe, and Australia published between 2009 and 2018. Eight studies reported direct costs and seven studies reported indirect costs. Direct costs varied from $3356 (Hungary) to $27 032 (Germany) with hospitalization and medication being two of the biggest components of direct medical costs in most studies. The indirect costs for lost productivity varied from $2433 (Italy) to $20 663 (UK), accounting for a significant portion of the total economic burden. CONCLUSIONS: Large variations existed in annual costs of SSc, but all studies found that SSc imposed a substantial economic burden on patients and their families. The identified studies were mostly from high-income countries and there is still a knowledge gap regarding the cost of SSc in other parts of the world.

Prevalence and incidence of systemic sclerosis: A systematic review and meta-analysis.

OBJECTIVE: Systemic sclerosis (SSc) is a rare debilitating autoimmune disease of the connective tissue. This study aimed to assess the recent prevalence and incidence of SSc across the world. METHODS: Using a systematic search strategy, PubMed/MEDLINE and EMBASE were searched to identify relevant studies published between 2006 and 2016. Two reviewers independently evaluated studies for inclusion based on inclusion/exclusion criteria and performed data extraction. The review was conducted and reported following the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement. The pooled prevalence of SSc was calculated by meta-analysis using a random-effects model. RESULTS: There were 1364 references retrieved using the initial searching strategy, and 20 epidemiological publications were selected for data extraction. The identified studies reported prevalence ranging from 3.8 per 100 000 in Taiwan to 50 per 100 000 in the USA. The prevalence was 23 per 100 000 (95% CI: 16-29 per 100 000; 18 studies) in a pooled sample of 11 574 individuals. Incidence rate of SSc ranges from 0.77 per 100 000 person-years in the Netherlands to 5.6 per 100 000 person-years in the USA. SSc predominates in females with higher prevalence and incidence rates. It is important to note that different methodologies were used to derive these numbers so comparisons were made with caution. CONCLUSION: This review provides an assessment of the current estimates of disease prevalence and incidence of SSc. The epidemiological burdens of SSc vary among different regions of the world. The epidemiological data need to be interpreted with caution considering the methodological differences in deriving these estimates.