% 0期刊文章%钱,李Junyan %, Mengtao%A Zhang, Xiao %A Wang, Qian %A Zhao, Jiuliang %A Tian, Zhuang %A Wei, Wei %A Zuo, Xiaoxia %A Zhang, Miaojia %A Zhu, Ping %A Ye, Shuang %A Zhang, Wei %A Zheng, Yi %A Qi, Wufang %A Li, Yang %A Zhang, Zhuoli %A Ding, Feng %A Gu, Jieruo %A Liu, Yi %A Wang, Yanhong %A Zeng, Xiaofeng %T Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study %D 2019 %R 10.1183/13993003.00081-2018 %J European Respiratory Journal %P 1800081 %V 53 %N 2 %X 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 %U //www.qdcxjkg.com/content/erj/53/2/1800081.full.pdf