Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

• Main Authors: Qian Wang (Author), Junyan Qian (Author), Mengtao Li (Author), Xiao Zhang (Author), Wei Wei (Author), Xiaoxia Zuo (Author), Ping Zhu (Author), Shuang Ye (Author), Wei Zhang (Author), Yi Zheng (Author), Wufang Qi (Author), Yang Li (Author), Zhuoli Zhang (Author), Feng Ding (Author), Jieruo Gu (Author), Yi Liu (Author), Can Huang (Author), Jiuliang Zhao (Author), Yongtai Liu (Author), Zhuang Tian (Author), Yanhong Wang (Author), Miaojia Zhang (Author), Xiaofeng Zeng (Author)
• Format: Book
• Published: SAGE Publishing, 2022-07-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Summary

Objective: This study evaluated the prognostic value of the multivariable risk assessment for systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH). Methods: A multicenter prospective cohort of SLE-associated PAH (CSTAR-PAH cohort) diagnosed based on right heart catheterization (RHC) was established. Baseline and follow-up records were collected. Three methods of risk assessment, including (1) the number of low-risk criteria, based on World Health Organization functional class (WHO FC), 6-min walking distance (6MWD), right atrial pressure (RAP), and cardiac index (CI); (2) the three-strata stratification based on the average risk score of four variables (WHO FC, 6MWD, RAP, and CI); and (3) the four-strata stratification based on COMPARE 2.0 model were applied. A risk-assessment method using three noninvasive low-risk criteria was applied at the first follow-up visit. Survival curves between patients with different risk groups were compared by Kaplan-Meier's estimation and log-rank test. Results: Three-hundred and ten patients were enrolled from 14 PAH centers. All methods of stratification at baseline and first follow-up significantly discriminated long-term survival. Survival rates were also significantly different based on the noninvasive risk assessment in first follow-up visit. Survival deteriorated with the escalation of risk from baseline to first follow-up. Patients with baseline serositis had a higher rate of risk improvement in their follow-up. Conclusion: The risk assessment has a significant prognostic value at both the baseline and first follow-up assessment of SLE-associated PAH. A noninvasive risk assessment can also be useful when RHC is not available during follow-up. Baseline serositis may be a predictor of good treatment response in patients with SLE-associated PAH.

Item Description

2040-6231
10.1177/20406223221112528