Long-term effects of selexipag in systemic sclerosis-associated digital ulcers: a case control multicentre observational study

Abstract

Objectives: Digital ulcers (DUs) affect ∼50% of systemic sclerosis (SSc) patients, causing significant pain and functional impairment. Current management involves both systemic and local therapies. However, the burden in terms of pain and quality of life due to refractory DUs still remains heavy. While selexipag is approved for SSc-associated pulmonary arterial hypertension, its potential in treating DUs is unexplored. We aimed to evaluate the long-term efficacy of selexipag compared with iloprost in treating DUs.

Methods: In this multicentre case-control study, we retrospectively evaluated 96 SSc patients with refractory DUs (32 treated with selexipag, 64 with iloprost), matched for gender, disease subset, and age. DU number, ischemic pain and Raynaud phenomenon (RP) severity were assessed at baseline, 6, 12, and 24 months. Pain and RP were evaluated using the Likert Pain Scale (LPS) and Raynaud Condition Score (RCS), respectively. Additionally, DUs recurrence and new onset were recorded. Healing rates were estimated using Kaplan-Meier analysis.

Results: Selexipag showed higher efficacy with 87% of DUs healing rate vs 28% for iloprost at 96 weeks (p< 0.001). DUs number, RCS, and LPS showed significant improvement in selexipag-treated patients compared with iloprost (p< 0.001 for all) throughout 24 months. Selexipag-treated patients achieved faster healing (75% by week 40) and maintained significantly lower relapse rates (5% vs 45% at 24 months, p< 0.001). New DUs formation remained consistently lower in the selexipag group compared with the iloprost group (5% vs 40% at 24 months, p< 0.001).

Conclusions: This observational study suggests that selexipag may be strongly effective in treating DUs refractory to conventional drugs.

Keywords: Raynaud condition score; Systemic sclerosis; digital ulcers; iloprost; relapse; selexipag; vasoactive therapy.