Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis
Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis
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DOI：
10.1093/rheumatology/kep107
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发表时间：
2009-06-01
期刊：
影响因子：
5.5
通讯作者：
Steen, V.
中科院分区：
文献类型：
--
作者：
McLaughlin, V.;Humbert, M.;Steen, V.
肺动脉高压(PAH)是许多CTD的破坏性血管并发症。在SSC患者中，PAH对预后和生存有重大影响，是与疾病相关的死亡的最常见原因。建议SSc患者每年进行一次PAH超声心动图筛查。怀疑右心插管确诊为PAH是必要的。PAH相关SSc(PAH-SSC)患者的治疗目标旨在减缓疾病进展和改善生活质量。用于评估PAH-SSC患者治疗效果的一些措施仍有待充分验证；例如，6分钟步行距离是评估运动能力的一种简单且可重复的手段，但需要了解这一特定患者群体的临床相关变化是什么。目前，对PAH-SSC的药物干预可能针对PAH-SSC的三条病理生理通路中的一条或多条。前列环素类似物依波前列烯醇已被证明可以改善PAH-SSC患者的运动能力和血流动力学，类似的数据可以从关于曲普替尼和伊洛前列素的较小规模研究中获得。双重内皮素受体拮抗剂波生坦已被证明可以改善PAH-SSC的运动能力和血流动力学，在接受内皮素A受体拮抗剂Sitaxsenan和Ambrisentan治疗的少数患者中也获得了类似的数据。用西地那非或他达拉非抑制磷酸二酯酶-5型可以解决一氧化氮产生受损的问题。多种靶向治疗的组合可能对这一患者群体有利。
Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of CTDs. In patients with SSc, PAH has a dramatic impact on prognosis and survival and is the single most common cause of disease-related death. Yearly echocardiographic screening for PAH is recommended in patients with SSc. It suspected, confirmation of PAH diagnosis by right heart catheterization is necessary. Treatment goals for patients with PAH associated with SSc (PAH-SSc) aim to slow disease progression and improve quality of life. Some measures used to gauge the effect of treatment in patients with PAH-SSc remain to be fully validated; the 6-min walk distance, for example, is a simple and reproducible means of assessing exercise capacity, but there exists a need to understand what constitutes a clinically relevant change in this specific patient population. Currently, pharmacological intervention in PAH-SSc may target one or more of three pathophysiological pathways in PAR The prostacyclin analogue epoprostenol has been shown to improve exercise capacity and haemodynamics in PAH-SSc patients and similar data are available from smaller studies on trepostinil and iloprost. The dual endothelin receptor antagonist bosentan has been shown to improve exercise capacity and haemodynamics in PAH-SSc, and similar data have been obtained in small numbers of patients treated with the endothelin receptor A antagonists sitaxsentan and ambrisentan. Impaired production of nitric oxide may be addressed by inhibiting phosphodiesterase type-5 with sildenafil or possibly tadalafil. Combinations of multiple targeted therapies may be beneficial to this patient population.