TY - T1的智慧患者的长期预后h systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00081-2018 VL - 53 IS - 2 SP - 1800081 AU - Qian, Junyan AU - Li, Mengtao AU - Zhang, Xiao AU - Wang, Qian AU - Zhao, Jiuliang AU - Tian, Zhuang AU - Wei, Wei AU - Zuo, Xiaoxia AU - Zhang, Miaojia AU - Zhu, Ping AU - Ye, Shuang AU - Zhang, Wei AU - Zheng, Yi AU - Qi, Wufang AU - Li, Yang AU - Zhang, Zhuoli AU - Ding, Feng AU - Gu, Jieruo AU - Liu, Yi AU - Wang, Yanhong AU - Zeng, Xiaofeng Y1 - 2019/02/01 UR - //www.qdcxjkg.com/content/53/2/1800081.abstract N2 - 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 ER -