Selexipag Therapy for Raynaud Phenomenon-induced Severe Digital Ischemia in Intravenous Epoprostenol Responders With Connective Tissue Disease

Raynaud phenomenon (RP) in connective tissue disease (CTD) can be resistant to oral vasodilator therapy, resulting uncontrollable digital ischemia. Intravenous (IV) prostanoids are often used, but the benefit transient and not all patients respond. Selexipag is an orally active selective IP-type prostanoid receptor agonist used pulmonary arterial hypertension. We describe 2 where selexipag significantly reduced ischemia after failure of other vasodilating agents. Subject 1 with undifferentiated CTD had impressive healing her fingers (Figure 1) subject systemic sclerosis (SSc) a …