Joglekar Amit, Tsai Fausan S, McCloskey Deborah A, Wilson Julianne E, Seibold James R, Riley David J
Division of Pulmonary and Critical Care Medicine, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
J Rheumatol. 2006 Jan;33(1):61-8.
. To assess the efficacy and tolerability of bosentan in pulmonary arterial hypertension secondary to systemic sclerosis (SSc-PAH) including patients with restrictive lung disease.
We retrospectively reviewed 23 SSc-PAH patients with PAH at baseline [PA systolic pressure (PASP) >or= 45 mm Hg by echocardiogram or mean PA pressure > 25 mm Hg at rest by cardiac catheterization], World Health Organization (WHO) functional classes II-IV, and with data available for 18 months. Bosentan dose was 62.5 mg twice daily for 1 month then 125 mg twice daily. Outcomes were WHO functional class, PASP, and pulmonary function tests (PFT) at 3-month intervals for 18 months.
WHO class at baseline 3.1 +/- 0.1 (mean +/- SE); 3 months, 2.5 +/- 0.2*; 6 months, 2.4 +/- 0.2*; 9 months, 2.5 +/- 0.2* (*p < 0.02 vs baseline, n = 21 to 23), indicating clinical improvement at 9 months. After 9 months, results were not significant versus baseline. Reduction in WHO class by at least one rank was 57% at 3 months; none worsened. After 9 months, WHO class tended to worsen compared to baseline. Baseline PASP was 54 +/- 2 mm Hg (n = 23) and did not change significantly with therapy. Restriction (total lung capacity 76% +/- 4% of predicted) and reduced diffusing capacity (39% +/- 3% of predicted) were unchanged during therapy. Abnormal transaminases in 2 patients (9%) necessitated discontinuing drug in both.
Bosentan is clinically beneficial in patients with SSc-PAH including patients with restrictive lung disease, but pulmonary hemodynamics and PFT results remained stable during treatment.
评估波生坦对系统性硬化症相关性肺动脉高压（SSc-PAH）患者（包括合并限制性肺病的患者）的疗效和耐受性。
我们回顾性分析了23例基线时患有PAH的SSc-PAH患者[经超声心动图检查肺动脉收缩压（PASP）≥45 mmHg，或经心导管检查静息时平均肺动脉压>25 mmHg]，世界卫生组织（WHO）功能分级为II-IV级，且有18个月可用数据。波生坦剂量为62.5 mg，每日两次，服用1个月，然后125 mg，每日两次。观察指标为18个月内每3个月一次的WHO功能分级、PASP和肺功能测试（PFT）。
基线时WHO分级为3.1±0.1（均值±标准误）；3个月时为2.5±0.2*；6个月时为2.4±0.2*；9个月时为2.5±0.2*（*与基线相比p<0.02，n=21至23），表明9个月时临床症状改善。9个月后，与基线相比结果无显著差异。3个月时WHO分级至少降低一级的比例为57%；无患者病情恶化。9个月后，与基线相比WHO分级有恶化趋势。基线时PASP为54±2 mmHg（n=23），治疗期间无显著变化。治疗期间，肺限制性通气功能障碍（肺总量为预测值的76%±4%）和弥散功能降低（为预测值的39%±3%）无变化。2例患者（9%）出现转氨酶异常，均需停药。
波生坦对SSc-PAH患者（包括合并限制性肺病的患者）具有临床益处，但治疗期间肺血流动力学和PFT结果保持稳定。
