Abstract
Objectives Primary Sjögren'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. 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, hazard ratio (HR) 0.161), pulmonary vascular resistance (p=0.016, HR 1.105) and Sjögren's syndrome disease damage index (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. Patient 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.
Abstract
The prognosis of primary Sjögren's syndrome-associated pulmonary arterial hypertension might be improved by improving reserved cardiopulmonary function, by achieving a damage-free state and especially by achieving low-risk category https://bit.ly/3h7mZ9h
Footnotes
This article has supplementary material available from erj.ersjournals.com
Author contributions: X. Zeng is the guarantor of this article. J. Wang, M. Li, X. Zhang, J. Qian, J. Zhao, Q. Wang and X. Zeng contributed to the study design and data analysis and interpretation. All authors contributed to the data collection. J. Wang, J. Zhao and Y. Wang contributed to the statistical analysis. J. Wang, M. Li and X. Zeng contributed to manuscript preparation. All authors gave their final approval of the manuscript.
Conflict of interest: J. Wang has nothing to disclose.
Conflict of interest: M. Li has nothing to disclose.
Conflict of interest: Q. Wang has nothing to disclose.
Conflict of interest: X. Zhang has nothing to disclose.
Conflict of interest: J. Qian has nothing to disclose.
Conflict of interest: J. Zhao has nothing to disclose.
Conflict of interest: D. Xu has nothing to disclose.
Conflict of interest: Z. Tian has nothing to disclose.
Conflict of interest: W. Wei has nothing to disclose.
Conflict of interest: X. Zuo has nothing to disclose.
Conflict of interest: M. Zhang has nothing to disclose.
Conflict of interest: P. Zhu has nothing to disclose.
Conflict of interest: S. Ye has nothing to disclose.
Conflict of interest: W. Zhang has nothing to disclose.
Conflict of interest: Y. Zheng has nothing to disclose.
Conflict of interest: W. Qi has nothing to disclose.
Conflict of interest: Y. Li has nothing to disclose.
Conflict of interest: Z. Zhang has nothing to disclose.
Conflict of interest: F. Ding has nothing to disclose.
Conflict of interest: J. Gu has nothing to disclose.
Conflict of interest: Y. Liu has nothing to disclose.
Conflict of interest: Y. Wang has nothing to disclose.
Conflict of interest: Y. Zhao has nothing to disclose.
Conflict of interest: X. Zeng has nothing to disclose.
Support statement: This work was supported by the Chinese National Key Research R&D Program (grant number 2017YFC0907601, 2017YFC0907602, 2017YFC0907605, 2008BAI59B02) and the Chinese National High Technology Research and Development Program, Ministry of Science and Technology (grant number 2012AA02A513). Funding information for this article has been deposited with the Crossref Funder Registry.
- Received November 7, 2019.
- Accepted June 3, 2020.
- Copyright ©ERS 2020