RT期刊文章SR电子T1长期掠夺nosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1800081 DO 10.1183/13993003.00081-2018 VO 53 IS 2 A1 Qian, Junyan A1 Li, Mengtao A1 Zhang, Xiao A1 Wang, Qian A1 Zhao, Jiuliang A1 Tian, Zhuang A1 Wei, Wei A1 Zuo, Xiaoxia A1 Zhang, Miaojia A1 Zhu, Ping A1 Ye, Shuang A1 Zhang, Wei A1 Zheng, Yi A1 Qi, Wufang A1 Li, Yang A1 Zhang, Zhuoli A1 Ding, Feng A1 Gu, Jieruo A1 Liu, Yi A1 Wang, Yanhong A1 Zeng, Xiaofeng YR 2019 UL //www.qdcxjkg.com/content/53/2/1800081.abstract AB This study aimed to identify the long-term clinical outcomes and prognostic factors of patients with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) confirmed by right heart catheterisation.A multicentre prospective cohort of SLE-associated PAH was established. Baseline and follow-up records were collected. The primary end-point was death. The secondary exploratory end-point was treatment goal achievement (TGA), defined as an integrated outcome.In total, 310 patients were enrolled from 14 PAH centres. The 1-, 3- and 5-year survival rates were 92.1%, 84.8% and 72.9%, respectively. The 1-, 3- and 5-year TGA rates were 31.5%, 53.6% and 62.7%, respectively. Baseline serositis, 6-min walking distance >380 m and cardiac index ≥2.5 L·min−1·m−2 were identified as independent prognostic factors of TGA. Patients with baseline serositis were more likely to reach TGA after intensive immunosuppressive therapy. TGA was identified as a positive predictor of survival in patients with SLE-associated PAH.TGA was associated with long-term survival, which supports the treat-to-target strategy in SLE-associated PAH. Baseline heart function predicted both survival and treatment goal achievement in patients with SLE-associated PAH. Patients with serositis at baseline tended to benefit from intensive immunosuppressive therapy and have a better clinical outcome.The long-term prognosis of SLE-associated PAH is newly reported by a large multicentre cohort study http://ow.ly/FRaS30mNAZA