Withdrawal of Epoprostenol Therapy in a Patient with Pulmonary Hypertension Associated with Sjögren's Syndrome

• Published in: Internal Medicine Vol. 53; no. 19; pp. 2237 - 2240
• Main Authors: Fujita, Tetsuo, Tanabe, Nobuhiro, Kasahara, Yasunori, Sugiura, Toshihiko, Sakao, Seiichiro, Tatsumi, Koichiro
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.01.2014
• Subjects: Administration, Oral; Adult; Antihypertensive Agents - administration & dosage; Antihypertensive Agents - therapeutic use; combination oral therapy; epoprostenol; Epoprostenol - administration & dosage; Epoprostenol - therapeutic use; Female; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Injections, Intravenous; pulmonary arterial hypertension; Sjogren's Syndrome - complications; Sjogren's Syndrome - diagnosis; Sjögren's syndrome; transition; Withholding Treatment
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.53.2885

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 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.