04 February 2019 by Junyan Qian

Composite goals plus inflammation: further risk assessment for SLE-PAH in CSTAR-PAH cohort


This study aimed to investigate the validity of the risk assessment and the prognostic value of it in Systemic lupus erythematosus (SLE)-PAH.Methods: All SLE patients were fulfilled the 1997 revised ACR criteria. PAH was diagnosed based on ESC/ERS guidelines by right heart catheterization. The outcome was all-cause mortality. Two different methods of risk categorization were applied according to baseline data, including low-risk criteria number of none to four and mean score of 1 (low-risk), 2 (intermediate-risk) or 3 (high-risk). According to first follow-up,patients were further divided into increased risk, remained risk and decreased risk group. A prediction model was used to distinguish SLE-PAH from vasculitic and vasculopathic subtype, based on the time interval between the diagnosis of SLE and PAH and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Kaplan–Meier survival curves and Cox proportional hazards analysis were conducted.


282 patients were enrolled. The 5-year survival of patients with none, one, two, three and four low risk criteria were 42.7%, 64.8%, 86.1%, 90.2% and 91.7%, respectively (HR=0.59, 95% CI 0.44-0.78, p<0.001). The 5-year survival of patients in low-risk, intermediate-risk and high-risk group were 92.3%, 60.4% and 50.0%, respectively (Log-rank, p=0.001). Notably, in low-risk group, patients with vasculitic subtype had better survival than those with vasculopathic subtype (Log-rank, p=0.044). The 5-year survival of patients with remained, decreased and increased risk were 65.4%, 88.1% and 23.8%, respectively (log-rank, p<0.001).


Our study validated the prognostic value of risk stratification strategy at baseline and follow-up visit in patients with SLE-PAH. The SLE disease activity and systemic manifestations predict the phenotype of SLE-PAH, which also effect the longterm survival and need to be involved into risk assessment of SLE-PAH. Improving to low-risk group can be a future treatment target for SLE-associated PAH patients in clinical practice.


About the author

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Junyan Qian


Peking Union Medical College Hospital


Key Contributors

Junyan Qian 1, Mengtao Li 1, Qian Wang 1, Jiuliang Zhao 1, Zhuang Tian 2, Wei Wei 3, Xiao Zhang 4, Xiaoxia Zuo 5, Miaojia Zhang 6, Ping Zhu 7, Shuang Ye 8, Wei Zhang 8, Yi Zheng 9, Wufang Qi 10, Yang Li 11, Zhuoli Zhang 12, Feng Ding 13, Jieruo Gu 14, Yi Liu 15, Xiaofeng Zeng 1 : 1 Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. 2 Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Beijing, China. 3 Department of Rheumatology, the General Hospital of TianJin Medical University, Tianjin, China. 4 Department of Rheumatology, Guangdong Provincial People’s Hospital, Guangzhou, China. 5 Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, China. 6 Department of Rheumatology, Jiangsu Provincial People’s Hospital, Nanjing, China. 7 Department of Rheumatology, Xijing Hospital Affiliated to the Fourth Military Medical University, Xi’an, China. 8 Department of Rheumatology, Ren Ji Hospital South Campus, Shanghai Jiao Tong University, Shanghai, China 9 Department of Rheumatology, Beijing Chao-Yang Hospital Affiliated to Capital Medical University, Beijing, China 10 Department of Rheumatology, Tianjin First Central Hospital, Tianjin, China. 11 Department of Rheumatology, the Second Affiliated Hospital of Harbin Medical University, Harbin, China. 12 Department of Rheumatology, Peking University First Hospital, Beijing, China. 13 Department of Rheumatology, Qilu Hospital of Shandong University, Jinan, China 14 Department of Rheumatology, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China 15 Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China

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