RT Journal Article
SR Electronic
T1 Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP erj01828-2013
DO 10.1183/09031936.00182813
A1 Yan-Jie Hao
A1 Xin Jiang
A1 Wei Zhou
A1 Yong Wang
A1 Lan Gao
A1 Yu Wang
A1 Guang-Tao Li
A1 Tao Hong
A1 Yong Huo
A1 Zhi-Cheng Jing
A1 Zhuo-Li Zhang
YR 2014
UL http://erj.ersjournals.com/content/early/2014/05/02/09031936.00182813.abstract
AB 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. Systemic lupus erythematosus is the most common underlying disease in Chinese patients with CTD-APAH http://ow.ly/vo4x6