A New Promising Role for Selexipag in the Treatment of Scleroderma Vasculopathy: Preliminary Results From a Third Level Italian Scleroderma Center

Aim Raynaud phenomenon (RP) and digital ulcers (DUs) represent the most frequent manifestations in systemic sclerosis (SSc), being signs of severe vasculopathy. Selexipag has been proposed as a useful tool for treating peripheral vasculopathy. The aim of this study was to evaluate the clinical effectiveness of Selexipag for treating complex cases of DUs as compared to the standard of care. Methods This is a case-control study in which SSc patients with active and long-lasting DUs were compared with a control group treated with a standard-of-care topical therapy that was stable in the previous 6 months. Clinical outcomes of RP and DUs were evaluated at T0 and after 6 months of follow-up. Results 23 SSc patients were enrolled. Selexipag was administered to 9 SSc patients, and its effects were compared with those in 14 SSc patients treated with standard-of- care therapy. Complete healing of DUs in Selexipag patients was higher (56% vs. 7%, p = 0.018). DUs diameter and pain VAS were slightly better-although not statistically significant-at the end of follow-up in the Selexipag group (respectively 0 vs. 5 p = 0.15 and 5 vs. 6, p = 0.066). All Raynaud Condition Scores outcomes improved in the Selexipag group: number of attacks (4 vs. 6, p < 0.001), duration (20 min vs. 25 mm, p = 0.083), and VAS (5 vs. 6, p = 0.066). Moreover, baseline DU diameter (beta = 0.70, p < 0.001) and Selexipag treatment (beta = -7.1, p = 0.006) were statistically significant predictors of the diameter of DUs after 6 months (F-test = 0.038). No new DUs were observed during the 6-month therapy. Conclusions Our preliminary results suggest a potentially new therapeutic indication of Selexipag in SSc for the treatment of SSc vasculopathy, in particular DUs and RP.