系统性硬化病相关肺动脉高压

• 作 者: 汪汉(综述)
• 机 构: 成都市心血管病研究所成都市第三人民医院心内科四川成都610031
• 出 处: 心血管病学进展
• 发表日期: 2017年1期
• 关键词: Systemic sclerosis Pulmonary arterial hypertension Connective tissue disease-ass

系统性硬化病相关肺动脉高压是结缔组织疾病相关肺动脉高压中最常见的一种类型，也是系统性硬化病的主要死亡原因，其病死率为特发性肺动脉高压的3～4倍，一经诊断，3年内病死率可达50％。因系统性硬化病常合并心肺、肌肉的并发症，肺动脉高压症状常常隐匿。6分钟步行试验、心脏彩色超声以及心脏磁共振成像检查对诊断系统性硬化病相关肺动脉高压有一定作用，但是，相对于特发性肺动脉高压而言，其治疗效果与预后均不佳。现就系统性硬化病相关肺动脉高压的流行病学、病因、发病机制、临床特点、诊断评估及治疗做一综述。“,”Systemic sclerosis-associated pulmonary arterial hypertension ( SSc-PAH) is the most common kind of connective tissue diseases-associated PAH,and is the leading cause of death in SSc.Compared with idiopathic PAH(IPAH),patients with SSc-PAH have a threefold increased risk of death ,with a 50%mortality rate within 3 years of PAH diagnosis .These patients often receive a late diagnosis be-cause of insidious onset and high prevalence of cardiac ,pulmonary ,and musculoskeletal co-morbidities .The current methods for screening and longitudinal evaluation of SSc-PAH are the 6-min walk test,transthoracic echocardiography ,and MRI.Unfortunately,patients with SSc-PAH receive a poor treatment and prognosis .