Clinical and LASCA preliminary data on selexipag efficacy for the treatment of digital vasculopathy in systemic sclerosis.

Abstract

Systemic sclerosis (SSc) is burdened by Raynaud's phenomenon (RP) and digital ulcers (DUs) and sometimes standard vasoactive therapies are ineffective or contraindicated. Selexipag is an oral selective IP prostacyclin receptor agonist approved for the treatment of SSc-related pulmonary arterial hypertension. We aimed to evaluate clinical and instrumental efficacy of selexipag in SSc digital vasculopathy. SSc patients with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies were administered selexipag, evaluating at baseline and after 3 months clinical outcomes regarding RP and DUs and digital perfusion assessed by laser speckle contrast analysis (LASCA). Selexipag was administered to 9 SSc patients (66.6% female, mean age 52.3 ±16.6 years). One of them had to stop the drug for adverse effects. After three months selexipag determined a significant reduction of RP daily episodes (p=0.012) and RP mean duration (p=0.044). DUs decreased from 10 to 4 without reaching the statistical significance. A significant improvement in the mean perfusion of the fingers (p=0.016) was observed at LASCA. Selexipag showed good potential for the treatment of SSc-digital vasculopathy. Our results are certainly preliminary but yet quite encouraging. New trials for the evaluation of selexipag efficacy in SSc-digital vasculopathy are needed.