波生坦长期治疗对系统性硬化症患者肺动脉高压发展的有益作用。
Beneficial effects of long-term treatment with bosentan on the development of pulmonary arterial hypertension in patients with systemic sclerosis.
作者信息
Murdaca Giuseppe, Lantieri Francesca, Puppo Francesco, Bezante Gian Paolo, Balbi Manrico
机构信息
Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Genoa, Italy
Department of Health Sciences, Biostatistics Unit, University of Genoa, Genoa, Italy.
出版信息
J Int Med Res. 2016 Sep;44(1 suppl):85-89. doi: 10.1177/0300060515593257.
OBJECTIVE
To investigate the effects of long-term treatment with bosentan on pulmonary arterial hypertension (PAH) in patients with systemic sclerosis.
METHODS
Patients with systemic sclerosis were followed between 2003 and 2014; those who developed digital ulcers were treated with standard regimens of bosentan. Patients were assessed at baseline and every 12 months using transthoracic Doppler echocardiography, 6-min walking distance test, Borg dyspnoea index and monitoring of plasma levels of 76-amino-acid N-terminal probrain natriuretic peptide. Patients who developed PAH underwent right heart catheterization to confirm the diagnosis.
RESULTS
Sixty-nine patients with systemic sclerosis were enrolled in the study. Of these, 25 developed digital ulcers and received treatment with bosentan; the remaining 44 comprised the control group. None of the patients treated with bosentan developed PAH during the follow-up period. Furthermore, in these patients the mean ± SD systolic pulmonary arterial pressure significantly decreased from 33.64 ± 2.91 mmHg at baseline to 26.20 ± 1.78 mmHg at the end of the follow-up period. In contrast, in the control group, seven patients developed PAH during the follow-up period, with the mean ± SD systolic pulmonary arterial pressure significantly increasing from 33.57 ± 2.75 mmHg at baseline to 39.41 ± 4.11 mmHg at the end of the follow-up period.
CONCLUSION
Long-term treatment with bosentan reduces the risk of developing PAH in patients with systemic sclerosis.
目的
探讨波生坦长期治疗对系统性硬化症患者肺动脉高压（PAH）的影响。
方法
对2003年至2014年期间的系统性硬化症患者进行随访；对出现指端溃疡的患者采用波生坦标准方案治疗。在基线期及之后每12个月，使用经胸多普勒超声心动图、6分钟步行距离测试、Borg呼吸困难指数以及监测76个氨基酸的N末端脑钠肽前体的血浆水平对患者进行评估。发生PAH的患者接受右心导管检查以确诊。
结果
69例系统性硬化症患者纳入本研究。其中，25例出现指端溃疡并接受波生坦治疗；其余44例组成对照组。在随访期间，接受波生坦治疗的患者均未发生PAH。此外，这些患者的平均收缩期肺动脉压（均值±标准差）从基线时的33.64±2.91 mmHg显著降至随访期末的26.20±1.78 mmHg。相比之下，对照组有7例患者在随访期间发生PAH，其平均收缩期肺动脉压从基线时的33.57±2.75 mmHg显著升至随访期末的39.41±4.11 mmHg。
结论
波生坦长期治疗可降低系统性硬化症患者发生PAH的风险。
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