PT - JOURNAL ARTICLE AU - Jieying Wang AU - Mengtao Li AU - Qian Wang AU - Xiao Zhang AU - Junyan Qian AU - Jiuliang Zhao AU - Dong Xu AU - Zhuang Tian AU - Wei Wei AU - Xiaoxia Zuo AU - Miaojia Zhang AU - Ping Zhu AU - Shuang Ye AU - Wei Zhang AU - Yi Zheng AU - Wufang Qi AU - Yang Li AU - Zhuoli Zhang AU - Feng Ding AU - Jieruo Gu AU - Yi Liu AU - Yanhong Wang AU - Yan Zhao AU - Xiaofeng Zeng TI - Pulmonary arterial hypertension associated with primary Sjogren's syndrome: a multi-centre cohort study from China AID - 10.1183/13993003.02157-2019 DP - 2020 Jan 01 TA - European Respiratory Journal PG - 1902157 4099 - http://erj.ersjournals.com/content/early/2020/06/08/13993003.02157-2019.short 4100 - http://erj.ersjournals.com/content/early/2020/06/08/13993003.02157-2019.full AB - Objectives Primary Sjogren's syndrome (pSS) is an important cause of pulmonary arterial hypertension (PAH), which remains insufficiently studied and needs attention. This study aimed to investigate the clinical characteristics, risk factors, prognosis and risk assessment of pSS-PAH.Methods We established a multicentre cohort of pSS-PAH diagnosed by right heart catheterisation (RHC). The case-control study was conducted with pSS-non PAH patients as a control group to identify the risk factors for PAH. In the cohort study, survival was calculated, and risk assessment was performed at both baseline and follow-up visits.Results In total, 103 patients with pSS-PAH were enrolled, with 526 pSS-non PAH patients as controls. The presence of anti-SSB (p<0.001, OR=4.095) and anti-U1RNP antibodies (p<0.001, OR=29.518), the age of pSS onset (p<0.001, OR=0.651) and the positivity of corneal staining (p=0.003, OR=0.409) were identified as independent risk factors for PAH. The 1-, 3- and 5-year survival rates were 94.0%, 88.8% and 79.0%, respectively. Cardiac index (p=0.010, HR=0.161), pulmonary vascular resistance (p=0.016, HR=1.105) and Sjogren's syndrome disease damage index (SSDDI, p=0.006, HR=1.570) were identified as potential predictors of death in pSS-PAH. Long-term outcomes were improved in patients in the low-risk category at baseline (p=0.002) and follow-up (p<0.0001).Conclusion The routine screening of PAH is suggested in pSS patients with early onset and positivity for anti-SSB or anti-U1RNP antibodies. Patients' prognosis might be improved by improving reserved cardiopulmonary function, by achieving a damage-free state and especially by achieving low-risk category, which supports the treat-to-target strategy for pSS-PAH.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Wang has nothing to disclose.Conflict of interest: Dr. Li has nothing to disclose.Conflict of interest: Dr. Wang has nothing to disclose.Conflict of interest: Dr. Zhang has nothing to disclose.Conflict of interest: Dr. Qian has nothing to disclose.Conflict of interest: Dr. Zhao has nothing to disclose.Conflict of interest: Dr. Xu has nothing to disclose.Conflict of interest: Dr. Tian has nothing to disclose.Conflict of interest: Dr. Wei has nothing to disclose.Conflict of interest: Dr. Zuo has nothing to disclose.Conflict of interest: Dr. Zhang has nothing to disclose.Conflict of interest: Dr. Zhu has nothing to disclose.Conflict of interest: Dr. Ye has nothing to disclose.Conflict of interest: Dr. Zhang has nothing to disclose.Conflict of interest: Dr. Zheng has nothing to disclose.Conflict of interest: Dr. Qi has nothing to disclose.Conflict of interest: Dr. Li has nothing to disclose.Conflict of interest: Dr. Zhang has nothing to disclose.Conflict of interest: Dr. Ding has nothing to disclose.Conflict of interest: Dr. Gu has nothing to disclose.Conflict of interest: Dr. Liu has nothing to disclose.Conflict of interest: Dr. Wang has nothing to disclose.Conflict of interest: Dr. Zhao has nothing to disclose.Conflict of interest: Dr. Zeng has nothing to disclose.