Pulmonary arterial hypertension (PAH) is a rare complication, but a significant prognostic factor in patients with Sjögren's syndrome (SjS). Despite its efficacy, the long-term use of intravenous epoprostenol is sometimes complicated by adverse effects, such as catheter-related infection. This case involves a 38-year-old woman with PAH associated with SjS (PAH-SjS) who was transitioned from treatment with long-term intravenous epoprostenol therapy to combination oral therapy containing bosentan

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... and tadalafil. She has remained in stable condition for more than two years following epoprostenol discontinuation. The details of this report suggest that long-term epoprostenol therapy can be safely tapered off and replaced with combination oral therapy in carefully selected patients with PAH-SjS.