Heresi Gustavo A, Minai Omar A
Department of Pulmonary, Allergy, and Critical Care Medicine, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
Respir Med. 2007 Oct;101(10):2099-107. doi: 10.1016/j.rmed.2007.05.020. Epub 2007 Jul 6.
Pulmonary hypertension (PH) is a serious complication of lupus. The effectiveness of current vasoactive therapy has not been well described.
Retrospective analysis of 12 patients with lupus-associated PH (age 43+/-10 years, mean+/-SD, all female) treated with pulmonary vasodilators.
At baseline, patients had severe PH: median six-minute walk distance (6MWD) 266 m (95% confidence interval [CI], 106 to 362); functional class III (n=7) and IV (n=5); mean pulmonary artery pressure (mPAP) 52 mmHg and cardiac index 2.23 L/min/m(2). Eight patients were started on epoprostenol and 2 each on bosentan or treprostinil. After a mean follow-up of 41+/-25 months, 5 patients were on combination therapy (3 epoprostenol plus bosentan, 1 treprostinil plus bosentan, 1 bosentan plus sildenafil) and 7 were on monotherapy (2 epoprostenol, 4 bosentan, 1 sildenafil); 6MWD increased by 139 m (95% CI, 36 to 259, p=0.007), 8 patients were functional class I or II and 4 were class III; right ventricular systolic pressure (RVSP) decreased by 22 mmHg (95% CI, 6 to 36; p=0.012), mPAP decreased by 18 mmHg (95% CI, 8 to 29; p=0.014), and cardiac index increased by 1.44 L/min/m(2) (95% CI, 0.76 to 2.08; p=0.016). There was no mortality or need for lung transplantation. Therapy was well tolerated.
Vasoactive therapy can achieve sustained clinical and hemodynamic improvement in lupus-associated PH.
肺动脉高压（PH）是狼疮的一种严重并发症。目前血管活性治疗的有效性尚未得到充分描述。
对12例接受肺血管扩张剂治疗的狼疮相关性PH患者（年龄43±10岁，平均±标准差，均为女性）进行回顾性分析。
基线时，患者患有严重的PH：六分钟步行距离（6MWD）中位数为266米（95%置信区间[CI]，106至362）；功能分级为III级（n = 7）和IV级（n = 5）；平均肺动脉压（mPAP）为52 mmHg，心脏指数为2.23 L/min/m²。8例患者开始使用依前列醇，2例分别使用波生坦或曲前列尼尔。平均随访41±25个月后，5例患者接受联合治疗（3例依前列醇加波生坦，1例曲前列尼尔加波生坦，1例波生坦加西地那非），7例接受单药治疗（2例依前列醇，4例波生坦，1例西地那非）；6MWD增加了139米（95% CI，36至259，p = 0.007），8例患者功能分级为I级或II级，4例为III级；右心室收缩压（RVSP）降低了22 mmHg（95% CI，6至36；p = 0.012），mPAP降低了18 mmHg（95% CI，8至29；p = 0.014），心脏指数增加了1.44 L/min/m²（95% CI，0.76至2.08；p = 0.016）。无死亡病例，也无需进行肺移植。治疗耐受性良好。
血管活性治疗可使狼疮相关性PH患者在临床和血流动力学方面持续改善。
