Long-term data on efficacy and safety of selexipag for scleroderma digital vasculopathy

• Published in: Journal of rheumatology
• Main Authors: Di Battista, Marco, Della Rossa, Alessandra, Mosca, Marta
• Format: Journal Article
• Language: English
• Published: Canada 15.06.2024
• ISSN: 1499-2752
• DOI: 10.3899/jrheum.2024-0103

Raynaud's phenomenon (RP) and digital ulcers (DUs) are the main signs of digital vasculopathy in systemic sclerosis (SSc). Selexipag is an oral prostacyclin agonist approved for SSc-related pulmonary arterial hypertension. Following our previous preliminary short-course report, we herein present long-term data on selexipag safety and efficacy in the treatment of SSc digital vasculopathy. Selexipag was administered to SSc patients with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies. Each subject was assessed at baseline and after 3, 6 and 12 months. Clinical outcomes related to RP and DUs were evaluated along with fingers skin score. Digital perfusion was assessed by laser speckle contrast analysis (LASCA). Nailfold videocapillaroscopy (NVC) was also performed. Eight SSc patients (63% female, mean age 50.1 years) received selexipag. After 12 months of treatment, RP was reported to significantly decrease in the number of daily episodes and mean duration (p<0.001 and p=0.01, respectively). All patients achieved a complete healing of their DUs (p=0.03) within six months. A progressive reduction of fingers skin score was observed (p=0.03). No structural changes of capillaries were noted at NVC. Conversely, LASCA revealed an important increase in total digital perfusion (p=0.004) despite seasonal variability. The safety profile was consistent with that reported in the literature. We observed a sustained efficacy of selexipag on SSc digital vasculopathy during one year of administration. Our promising results encourage the design of a new randomized controlled trial to evaluate the effect of selexipag on SSc digital vasculopathy.