Environmental and occupational factors in the aetiopathogenesis of systemic sclerosis: insights from a large multinational patient survey

Objective: Systemic sclerosis (SSc) is a rare and heterogeneous disease. SSc pathobiology is complex and it occurs in a genetically susceptible host. Our aim was to describe potential environmental and occupational exposures relevant to SSc. Method: An online survey was developed in English to collect detailed information about relevant current/past environmental and occupational exposures. Individuals with SSc were invited to participate through international patient associations and social media. Participation was voluntary and no identifiable information was collected. We used descriptive statistics to summarize our data. Results: In total, 478 evaluable individuals (93% female; 81% ≥ 51 years old) from 19 countries [mostly USA (68%) and UK (23%)] completed the survey. Concerning SSc subtype, 137 had diffuse and 178 limited cutaneous SSc; and around one-fifth mixed connective tissue disease/SSc overlap (n = 84). The majority had a long-established diagnosis of SSc (>10 years 50%, 5–10 years 20%). One-third were in active employment (full-time 23%, part-time 10%), 4.6% were self-employed, and 42% were retired. The most frequently reported current and past work-related exposures (reported by >10% of participants) included outdoor sun exposure, paint/varnishes/lacquers, weedkillers, wet work/cleaning agents, paint thinners/removers, biological agents, pesticides, solvents/degreasers, and ionizing radiation. Exposures previously implicated in SSc were also reported, including silica/quartz, PVC/plasticizers, vinyl chloride, and epoxy resins, although less frequently. Conclusio: We benchmark potential environmental and occupational factors in SSc. A novel finding was the identification of several important potential industrial/chemical agent exposures. These data could inform future SSc research concerning occupational/environmental triggers.