TY - JOUR T1 - Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients JF - European Respiratory Journal JO - Eur Respir J SP - 963 LP - 972 DO - 10.1183/09031936.00182813 VL - 44 IS - 4 AU - Yan-Jie Hao AU - Xin Jiang AU - Wei Zhou AU - Yong Wang AU - Lan Gao AU - Yu Wang AU - Guang-Tao Li AU - Tao Hong AU - Yong Huo AU - Zhi-Cheng Jing AU - Zhuo-Li Zhang Y1 - 2014/10/01 UR - http://erj.ersjournals.com/content/44/4/963.abstract N2 - 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 ER -