Self-reported systemic sclerosis-related symptoms are more prevalent in subjects with Raynaud’s phenomenon in the general population

Objective: Puffy fingers and Raynaud’s phenomenon (RP) are important predictors of early systemic sclerosis (SSc). Pulmonary involvement, which usually occurs in early disease, is the leading cause of mortality in patients with SSc. Furthermore, skin autofluorescence (SAF) might play a role in the early detection of SSc. We aim to assess the prevalence of SSc-related symptoms and SAF with a main focus on pulmonary complications in subjects with RP.
Methods: We analysed data from a large population-based cohort study. Participants with known connective tissue disease (CTD) were excluded. Patient characteristics, self-reported pulmonary problems, and spirometry were obtained. The CTD questionnaire provided information on SSc-related symptoms (i.e., RP, puffy fingers, distal/proximal skin thickening, and finger-tip lesions). Participants were categorized into definite RP (n=3911) and non-RP (n=70037). 
Results: In the total cohort (n=73948),  prevalence of at least one SSc-related symptom (other than RP) was 8.7%. Prevalence of SSc-related symptoms was 23.5% in subjects with RP, and 7.1% without RP (p<0.001). Distal skin thickening was the most prevalent symptom in both groups. Participants with RP and SSc-related symptoms more frequently reported dyspnoea at rest, dyspnoea after exertion and pulmonary fibrosis, and had the lowest mean forced vital capacity as compared to the other groups (all p<0.001). In multivariate regression, dyspnoea at rest/on exertion remained associated with increased risk of SSc-related symptoms in subjects with RP (both p<0.001). SAF was higher in the group of subjects with RP and SSc-related symptoms compared to the other groups (p<0.001).
Conclusion: Prevalence of SSc-related symptoms was approximately three-fold higher in subjects with RP. Pulmonary problems are more prevalent in subjects with RP who also reported SSc-related symptoms. This suggests that (suspected) early SSc develops more insidiously than acknowledged, warranting further research on early detection.