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.
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