Intravenous epoprostenol treatment of patients with connective tissue disease and pulmonary arterial hypertension at a single center
Intravenous epoprostenol treatment of patients with connective tissue disease and pulmonary arterial hypertension at a single center
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单中心静脉注射依前列醇治疗结缔组织病合并肺动脉高压患者
DOI：
10.3109/s10165-012-0828-1
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发表时间：
2013
影响因子：
2.2
通讯作者：
M. Kuwana
中科院分区：
文献类型：
--
作者：
Y. Shirai;H. Yasuoka;T. Takeuchi;T. Satoh;M. Kuwana
目的评价环丙沙星对日本结缔组织病(CTD)合并肺动脉高压(PAH)患者的治疗效果。方法2000-2009年间，16例PAH-CTD患者在同一中心持续静脉注射环丙沙星。评估了基线特征、短期和长期结果、死亡率的预测因素和安全性。结果6例系统性红斑狼疮患者，5例混合性CTD患者，4例系统性硬化症患者，1例原发性干燥综合征患者。治疗前6个月，平均肺动脉压(MPAP)、心脏指数(CI)、肺血管阻力和功能分级均有显著改善。治疗组1、2、3年的累积生存率分别为69、69、55%，明显优于对照组。功能分级、基线时的CI和6个月时mPAP的降低被确定为生存的预测因素。不良事件频繁发生，包括潮红和导管相关感染，但所有患者都能耐受治疗。结论根据我们的患者队列中短期和长期结果的改善，环前列腺素是治疗CTD合并晚期PAH的有效药物。
ObjectiveTo assess the efficacy of epoprostenol treatment in Japanese patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD).MethodsSixteen patients with PAH–CTD treated with continuous intravenous epoprostenol at a single center between 2000 and 2009 were enrolled. Baseline characteristics, short-term and long-term outcomes, predictors of mortality, and safety profiles were evaluated. For survival analysis, 16 controls were selected who matched the underlying CTD, World Health Organization functional class, and use of PAH drugs, except for epoprostenol.ResultsSix patients had systemic lupus erythematosus, five had mixed CTD, four had systemic sclerosis, and one had primary Sjögren’s syndrome. The mean pulmonary arterial pressure (mPAP), cardiac index (CI), pulmonary vascular resistance, and functional class were significantly improved during the first 6 months of epoprostenol treatment. Cumulative survival rates at 1, 2, and 3 years in epoprostenol-treated patients were 69, 69, and 55 %, respectively, and were significantly better than those of the controls. Functional class, CI at baseline, and reduction of mPAP at 6 months were identified as predictors of survival. Adverse events, including flushing and catheter-related infection, were frequent, but all patients tolerated the treatment.ConclusionBased on the improvements in both short-term and long-term outcomes among our patient cohort, epoprostenol is an effective treatment for CTD patients with advanced PAH.
影响因子：
9.6
作者：
Chung, Lorinda;Liu, Juliana;Zamanian, Roham T.
通讯作者：
Zamanian, Roham T.