Systemic Sclerosis-Associated Pulmonary Arterial Hypertension
Systemic Sclerosis-Associated Pulmonary Arterial Hypertension
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DOI：
10.1378/chest.12-2396
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发表时间：
2013-10-01
期刊：
影响因子：
9.6
通讯作者：
Hassoun, Paul M.
中科院分区：
文献类型：
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作者：
Chaisson, Neal F.;Hassoun, Paul M.
肺动脉高压（PAH）是导致系统性硬化症（SSc）死亡的主要原因，影响了多达12%的SSc患者，在PAH诊断后3年内死亡率为50%。与特发性多环芳烃（IPAH）相比，ssc相关性多环芳烃（SSc-PAH）患者的死亡风险增加了三倍，并且由于隐匿性发病和心脏、肌肉骨骼和肺组织共病的高患病率，可能在病程晚期才得到诊断。与IPAH队列相比，传统形式的PAH治疗通常效果较差；不幸的是，这背后的确切原因仍然知之甚少，但可能包括病理机制的变化，心血管对增加后负荷的反应的差异，以及在病程早期检测和治疗SSc-PAH的策略不足。目前用于SSc-PAH筛查和纵向评价的方法，如6分钟步行试验、经胸超声心动图和MRI，各有明显的优缺点。我们对SSc-PAH及其与IPAH的区别进行了最新的重点综述，包括发病机制、发病的适当筛查、治疗的新方法和对该疾病的纵向评估。
Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc) and affects up to 12% of all patients with SSc, with a 50% mortality rate within 3 years of PAH diagnosis. Compared with the idiopathic form of PAH (IPAH), patients with SSc-associated PAH (SSc-PAH) have a threefold increased risk of death and may receive a diagnosis late in the course of disease because of insidious onset and the high prevalence of cardiac, musculoskeletal, and pulmonary parenchymal comorbidities. Treatment with conventional forms of PAH therapy often yield poor results compared with IPAH cohorts; unfortunately, the exact reasons behind this remain poorly understood but likely include variations in the pathologic mechanisms, differences in cardiovascular response to increasing afterload, and inadequate strategies to detect and treat SSc-PAH early in its course. Current methods for screening and longitudinal evaluation of SSc-PAH, such as the 6-min walk test, transthoracic echocardiography, and MRI, each have notable advantages and disadvantages. We provide an up-to-date, focused review of SSc-PAH and how it differs from IPAH, including pathogenesis, appropriate screening for disease onset, and new approaches to treatment and longitudinal assessment of this disease.