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Pulmonary arterial hypertension associated with primary Sjogren's syndrome: a multi-centre cohort study from China.
Eur Respir J. 2020 Jul 02 [Online ahead of print]ER

Abstract

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.

Authors+Show Affiliations

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. Department of Rheumatology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China. contributed equally to this study.Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China mengtao.li@cstar.org.cn zengxfpumc@163.com. contributed equally to this study.Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. contributed equally to this study.Department of Rheumatology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China. contributed equally to this study.Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.Department of Rheumatology, Tianjin Medical University General Hospital, Tianjin, China.Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, China.Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China.Department of Clinical Immunology, PLA Specialized Research Institute of Rheumatology & Immunology, Xijing Hospital, Fourth Military Medical University, Xi'an, China.Department of Rheumatology, Ren Ji Hospital South Campus, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.Department of Rheumatology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.Department of Rheumatology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.Department of Rheumatology, the First Central Hospital, Tianjin, China.Department of Rheumatology, the Second Affiliated Hospital of Harbin Medical University, Harbin, China.Department of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China.Department of Rheumatology, Qilu Hospital of Shandong University, Jinan, China.Department of Rheumatology, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China.Department of Epidemiology and Bio-statistics, Institute of Basic Medical Sciences, China Academy of Medical Sciences & Peking Union Medical College, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China mengtao.li@cstar.org.cn zengxfpumc@163.com.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32616590

Citation

Wang, Jieying, et al. "Pulmonary Arterial Hypertension Associated With Primary Sjogren's Syndrome: a Multi-centre Cohort Study From China." The European Respiratory Journal, 2020.
Wang J, Li M, Wang Q, et al. Pulmonary arterial hypertension associated with primary Sjogren's syndrome: a multi-centre cohort study from China. Eur Respir J. 2020.
Wang, J., Li, M., Wang, Q., Zhang, X., Qian, J., Zhao, J., Xu, D., Tian, Z., Wei, W., Zuo, X., Zhang, M., Zhu, P., Ye, S., Zhang, W., Zheng, Y., Qi, W., Li, Y., Zhang, Z., Ding, F., ... Zeng, X. (2020). Pulmonary arterial hypertension associated with primary Sjogren's syndrome: a multi-centre cohort study from China. The European Respiratory Journal. https://doi.org/10.1183/13993003.02157-2019
Wang J, et al. Pulmonary Arterial Hypertension Associated With Primary Sjogren's Syndrome: a Multi-centre Cohort Study From China. Eur Respir J. 2020 Jul 2; PubMed PMID: 32616590.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pulmonary arterial hypertension associated with primary Sjogren's syndrome: a multi-centre cohort study from China. AU - Wang,Jieying, AU - Li,Mengtao, AU - Wang,Qian, AU - Zhang,Xiao, AU - Qian,Junyan, AU - Zhao,Jiuliang, AU - Xu,Dong, 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 - Zhao,Yan, AU - Zeng,Xiaofeng, Y1 - 2020/07/02/ PY - 2019/11/07/received PY - 2020/06/03/accepted PY - 2020/7/4/entrez JF - The European respiratory journal JO - Eur. Respir. J. N2 - 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. SN - 1399-3003 UR - https://www.unboundmedicine.com/medline/citation/32616590/Pulmonary_arterial_hypertension_associated_with_primary_Sjogren's_syndrome:_a_multi-centre_cohort_study_from_China L2 - http://erj.ersjournals.com/cgi/pmidlookup?view=long&amp;pmid=32616590 DB - PRIME DP - Unbound Medicine ER -
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