Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study. Issue 2 (14th February 2019)

• Record Type: Journal Article
• Authors: Qian, Junyan; Li, Mengtao; Zhang, Xiao; Wang, Qian; Zhao, Jiuliang; Tian, Zhuang; Wei, Wei; Zuo, Xiaoxia; Zhang, Miaojia; Zhu, Ping; Ye, Shuang; Zhang, Wei; Zheng, Yi; Qi, Wufang; Li, Yang; Zhang, Zhuoli; Ding, Feng; Gu, Jieruo; Liu, Yi; Wang, Yanhong; Zeng, Xiaofeng
• Other Names: author non-byline.; Huang C. author non-byline.; Yang X. author non-byline.; Xu D. author non-byline.; Liu Y. author non-byline.; Guo X. author non-byline.; Wang H. author non-byline.; Lai J. author non-byline.; Zhang N. author non-byline.; Yang K. author non-byline.; Liu Y. author non-byline.; Dong S. author non-byline.; Lei Y. author non-byline.; Feng Y. author non-byline.; Li Y. author non-byline.; Zhou Y. author non-byline.; Wang Q. author non-byline.; Jia J. author non-byline.; Han Q. author non-byline.; Guo L. author non-byline.; Chen J. author non-byline.; Zhang Y. author non-byline.; Liu Y. author non-byline.; Xu H. author non-byline.; Sun Y. author non-byline.; Hao Y. author non-byline.; Fan Y. author non-byline.; Shu Y.Q. author non-byline.; Lin Z. author non-byline.; Qing P. author non-byline.
• Is Part Of: European respiratory journal. Volume 53:Issue 2(2019)
• Publication Date: 2019-02-14
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.00081-2018
• Languages: English
• ISSNs: 0903-1936

Abstract

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