TY - JOUR T1 -系统性红斑狼疮相关肺动脉高压患者的长期预后:CSTAR-PAH队列研究摩根富林明-欧洲呼吸杂志》乔和J - 10.1183/13993003.00081 -2018欧元六世- 53 - 2 SP - 1800081 AU -钱,Junyan AU - Li Mengtao AU -张小盟——王黔盟——赵Jiuliang盟——田,壮族非盟-魏,魏盟-左,庐山逍夏盟,张Miaojia盟——朱,平盟——你们双盟——张AU - Zheng, AU - Yi, AU - Li, AU - Yang, AU - Zhang, AU - Ding, AU - Feng, AU - Gu, AU - jie, AU - Liu, AU - Yi, AU - Wang, AU - Zeng，Xiaofeng Y1 - 2019/02/01 UR - //www.qdcxjkg.com/content/53/2/1800081.abstract N2 -本研究旨在确定经右心导管术证实的系统性红斑狼疮(SLE)相关肺动脉高压(PAH)患者的长期临床结局和预后因素。建立了sle相关PAH的多中心前瞻性队列研究。收集基线和随访记录。主要终点是死亡。第二个探索性终点是治疗目标达成(TGA)，定义为综合结局。总共有310名患者来自14个PAH中心。1年、3年、5年生存率分别为92.1%、84.8%、72.9%。1年、3年和5年TGA率分别为31.5%、53.6%和62.7%。基线浆膜炎、6分钟步行距离380 m和心脏指数≥2.5 L·min−1·m−2被确定为TGA的独立预后因素。 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 -