|Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients|
|Hao, Yan-Jie1; Jiang, Xin2,3; Zhou, Wei1; Wang, Yong4; Gao, Lan1; Wang, Yu1; Li, Guang-Tao1; Hong, Tao5; Huo, Yong5; Jing, Zhi-Cheng2,3; Zhang, Zhuo-Li1|
|刊名||EUROPEAN RESPIRATORY JOURNAL|
|WOS标题词||Science & Technology|
|关键词[WOS]||SYSTEMIC-LUPUS-ERYTHEMATOSUS ; INTERSTITIAL LUNG-DISEASE ; HONG-KONG CHINESE ; TAKAYASU ARTERITIS ; CLINICAL CHARACTERISTICS ; RHEUMATIC-DISEASES ; REVISED CRITERIA ; SCLEROSIS ; SURVIVAL ; EPIDEMIOLOGY|
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.
|作者单位||1.Peking Univ, Hosp 1, Dept Rheumatol & Clin Immunol, Beijing 100034, Peoples R China|
2.Fu Wai Hosp, Peking Union Med Coll, Thrombosis & Vasc Med Ctr, State Key Lab Cardiovasc Dis, Beijing, Peoples R China
3.Chinese Acad Med Sci, Beijing 100730, Peoples R China
4.Capital Med Univ, Beijing Shijitan Hosp, Dept Pulm Vasc Dis, Beijing, Peoples R China
5.Peking Univ, Hosp 1, Dept Cardiol, Beijing 100034, Peoples R China
|Hao, Yan-Jie,Jiang, Xin,Zhou, Wei,et al. Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients[J]. EUROPEAN RESPIRATORY JOURNAL,2014,44(4):963-972.|
|APA||Hao, Yan-Jie.,Jiang, Xin.,Zhou, Wei.,Wang, Yong.,Gao, Lan.,...&Zhang, Zhuo-Li.(2014).Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients.EUROPEAN RESPIRATORY JOURNAL,44(4),963-972.|
|MLA||Hao, Yan-Jie,et al."Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients".EUROPEAN RESPIRATORY JOURNAL 44.4(2014):963-972.|