Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients
Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients
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DOI：
10.1183/09031936.00182813
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发表时间：
2014-10-01
影响因子：
24.3
通讯作者：
Zhang, Zhuo-Li
中科院分区：
文献类型：
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作者：
Hao, Yan-Jie;Jiang, Xin;Zhang, Zhuo-Li
为探讨中国结缔组织病相关肺动脉高压患者的特点、生存及死亡的危险因素，我们收集了5年来中国医院3个转诊中心之一就诊的129例经右心导管确诊的结缔组织病相关肺动脉高压患者。研究的终点是全因死亡或数据审查。系统性红斑狼疮是最常见的CTD(49%)，系统性硬化症仅占6%。1、3年总生存率分别为92%和80%。在研究人群中，心包积液、步行6分钟距离较短、混合静脉血氧饱和度较低、肺血管阻力(PVR)和碱性磷酸酶(ALP)较高以及总胆固醇水平较低均与较高的死亡风险相关。结论：与西方患者不同，系统性红斑狼疮是中国CTD-APAH患者最常见的基础疾病。中国CTD-APAH患者在现代治疗时代的存活率与西方国家相似。PVR和ALP升高是预后不良的独立危险因素。
We sought to investigate the characteristics, survival and risk factors for mortality in Chinese patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) in modern therapy era.129 consecutive adult patients who visited one of three referral centres in China with a diagnosis of CTD-APAH confirmed by right heart catheterisation during the previous 5 years were enrolled. The end-point was all-cause death or data censoring.Systemic lupus erythematosus was the most common underlying CTD (49%) and systemic sclerosis just accounted for 6% in this cohort. The overall survival at 1 and 3 years was 92% and 80%, respectively. Pericardial effusion, a shorter 6-min walk distance, lower mixed venous oxygen saturation, higher pulmonary vascular resistance (PVR) and alkaline phosphatase (ALP), and lower total cholesterol levels were all associated with a higher risk of death among the study population. Higher PVR and ALP were independent predictors of mortality.In conclusion, unlike in western patients, systemic lupus erythematosus is the most common underlying disease in Chinese patients with CTD-APAH. The survival of Chinese patients with CTD-APAH in the modern treatment era is similar to that in western countries. Elevated PVR and ALP are independent risk factors for poor outcomes.