title: Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients.
abstract: 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.
authors.affiliation: Dept of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China.
authors.forename: Yan-Jie
authors.initials: YJ
authors.lastname: Hao
authors.affiliation: Thrombosis and Vascular Medicine Center, State Key Lab of Cardiovascular disease, Fu Wai Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Beijing, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Jiang
authors.affiliation: Dept of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhou
authors.affiliation: Dept of Pulmonary Vascular disease, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Dept of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China.
authors.forename: Lan
authors.initials: L
authors.lastname: Gao
authors.affiliation: Dept of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China.
authors.forename: Yu
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Dept of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China.
authors.forename: Guang-Tao
authors.initials: GT
authors.lastname: Li
authors.affiliation: Dept of Cardiology, Peking University First Hospital, Beijing, China.
authors.forename: Tao
authors.initials: T
authors.lastname: Hong
authors.affiliation: Dept of Cardiology, Peking University First Hospital, Beijing, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Huo
authors.affiliation: Thrombosis and Vascular Medicine Center, State Key Lab of Cardiovascular disease, Fu Wai Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Beijing, China.
authors.forename: Zhi-Cheng
authors.initials: ZC
authors.lastname: Jing
authors.affiliation: Dept of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China zhuoli.zhang@126.com.
authors.forename: Zhuo-Li
authors.initials: ZL
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D044466:Asian People; D003240:Connective Tissue Diseases / Q000150:complications*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D055815:Young Adult