Fujita Tetsuo, Tanabe Nobuhiro, Kasahara Yasunori, Sugiura Toshihiko, Sakao Seiichiro, Tatsumi Koichiro
Department of Respirology, Graduate School of Medicine, Chiba University, Japan.
Intern Med. 2014;53(19):2237-40. 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.
肺动脉高压（PAH）是一种罕见的并发症，但却是干燥综合征（SjS）患者的一个重要预后因素。尽管静脉注射依前列醇有疗效，但长期使用有时会出现不良反应，如导管相关感染。本病例涉及一名38岁患有与干燥综合征相关的肺动脉高压（PAH-SjS）的女性，她从长期静脉注射依前列醇治疗过渡到含波生坦和他达拉非的联合口服治疗。在停用依前列醇后，她已保持稳定状态超过两年。本报告的细节表明，在精心挑选的PAH-SjS患者中，长期依前列醇治疗可以安全地逐渐减量并替换为联合口服治疗。
