Risk factors for the development of systemic sclerosis: a systematic review of the literature

Rheumatol Adv Pract. 2018 Oct 11;2(2):rky041. doi: 10.1093/rap/rky041. eCollection 2018.


Objectives: Although numerous studies have investigated the roles of various genetic, epigenetic and environmental factors that may impact its aetiology, SSc is still regarded as an idiopathic disease. Given that there is significant heterogeneity in what has been proposed to influence the development of SSc, this systematic review was conducted to assess the impacts of different factors on the aetiology of scleroderma.

Methods: The search was performed in the PubMed, CINAHL and SCOPUS databases on 17 May 2017. Any study that made explicit reference to scleroderma or SSc that had information about the risk factors or epidemiology of the disease was included. The extracted outcome variables were prevalence, gender preponderance, geographical distribution, family history and various proposed environmental risk factors.

Results: One thousand five hundred and seventy-four articles were screened for eligibility. Thirty-four articles were eligible for the systematic literature review.

Conclusion: Age between 45 and 64 years, female sex, positive family history and exposure to silica were found to be risk factors. There were conflicting findings regarding the impact of exposure to organic solvents and microchimerism. No relationship between infectious agents, alcohol consumption or cigarette smoking and the development of SSc was identified.

Keywords: epidemiology; risk factors; scleroderma; systemic sclerosis.