Predictors of progression to systemic sclerosis: analysis of very early diagnosis of systemic sclerosis in a large single-centre cohort

Objective This study analysed the very early disease of SSc (VEDOSS) characteristics in a group of 217 patients with RP and at least one manifestation of SSc in search of predictors for the progression to SSc. Methods This was a cross-sectional single-centre analysis of patients presenting with RP with a specific SSc clinical manifestation or SSc autoantibody or SD pattern at nailfold capillaroscopy (SD-NFC), without skin involvement, who attended a scleroderma outpatient clinic between 2010 and 2019. The performance of VEDOSS and the importance of the combination of VEDOSS characteristics to predict the progression to SSc were evaluated. Results Among 217 patients, 153 (70.5%) were classified as SSc, including 65 (30%) in the first investigation; 69.3% of the SSc patients met VEDOSS criteria compared with 6.3% of patients who did not progress to SSc. The combinations most associated with progression to SSc were RP + puffy fingers (PF) + positive ANA + SD-NFC and/or SSc-specific antibody (VEDOSS level 2), with an odds ratio (OR) of 19.52 (95% CI 4.48, 85.06; P < 0.001) and RP + PF + positive ANA (VEDOSS level 1; 'red flags') (OR 15.45; P < 0.001), while combinations without non-RP clinical symptoms, as RP + SD-NFC (OR 0.03; P < 0.001) and RP + anticentromere + SD-NFC (OR 0.06; P = 0.006) were associated with non-progression to SSc. Conclusion Among patients with RP with at least one manifestation of SSc, without skin involvement, combinations of VEDOSS characteristics were the strongest predictors of progression to SSc at a median follow-up of 4 years.

Automated summary

This study analyzed the early characteristics of SSc in RP patients and identified predictors for the progression to SSc. Combinations of RP + PF + positive ANA + SD-NFC and/or SSc-specific antibody (VEDOSS level 2), as well as RP + PF + positive ANA (VEDOSS level 1; "red flags") were most associated with progression to SSc. Combinations without non-RP clinical symptoms, such as RP + SD-NFC and RP + anticentromere + SD-NFC, were associated with non-progression to SSc.