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. 2021 Mar 4:8:618486.
doi: 10.3389/fmed.2021.618486. eCollection 2021.

Validation of the REVEAL Prognostic Models in Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension

Jingge Qu  1 , Mengtao Li  1 , Xiaofeng Zeng  1 , Xiao Zhang  2 , Wei Wei  3 , Xiaoxia Zuo  4 , Ping Zhu  5 , Shuang Ye  6 , Wei Zhang  6 , Yi Zheng  7 , Wufang Qi  8 , Yang Li  9 , Zhuoli Zhang  10 , Feng Ding  11 , Jieruo Gu  12 , Yi Liu  13 , Miaojia Zhang  14 , Junyan Qian  1 , Can Huang  1 , Jiuliang Zhao  1 , Qian Wang  1 , Yongtai Liu  15 , Zhuang Tian  15 , Yanhong Wang  16
Affiliations

Validation of the REVEAL Prognostic Models in Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension

Jingge Qu et al. Front Med (Lausanne). .

Abstract

No previous studies have investigated the predictive performance of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) prognostic equation and simplified risk score calculator in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH). We aimed to validate these prediction tools in an external cohort of patients with SLE-PAH. In this study, the validation cohort consisted of patients with SLE-PAH registered in a prospective, multicenter, nationwide database between November 2006 and May2016. The follow-up of patients was censored at 1 year. Discrimination, calibration, model fit, and risk stratification of the REVEAL prognostic equation and simplified risk score calculator were validated. As a result, a total of 306 patients with SLE-PAH were included. The 1-year overall survival rate was 91.5%. The C-index of the prognostic equation was 0.736, demonstrating reasonably good discrimination, and it was greater than that for the simplified risk score calculator (0.710). The overall calibration slope was 0.83, and the Brier score was 0.079. The risk of renal insufficiency and World Health Organization Functional Class III (WHO FC III) were underestimated, and the risk assigned to a heart rate >92 bpm in the REVEAL prognostic models was not observed in our validation cohort. Both model discrimination and calibration were poor in the very high-risk group. In conclusion, the REVEAL models exhibit good discriminatory ability when predicting 1-year overall survival in patients with SLE-PAH. Findings from both models should be interpreted with caution in cases of renal insufficiency, WHO FC III, and heart rate >92 bpm.

Keywords: 1-year survival; REVEAL model; pulmonary arterial hypertension; risk stratification; systemic lupus erythematosus.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Survival by risk group according to the prognostic equation (A) and simplified risk calculator (B).
Figure 2
Figure 2
Calibration plots in the risk groups stratified by the REVEAL prognostic equation (A) and simplified risk calculator (B). REVEAL, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management.
See this image and copyright information in PMC

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References

    1. Zhao J, Wang Q, Liu Y, Tian Z, Guo X, Wang H, et al. . Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: a cohort study in China. Int J Cardiol. 236, 432-437. (2017 Jun 1). 10.1016/j.ijcard.2017.01.097 - DOI - PubMed
    1. Hao YJ, Jiang X, Zhou W, Wang Y, Gao L, Wang Y, et al. . Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients. Eur Respir J. (2014) 44:963–72. 10.1183/09031936.00182813 - DOI - PubMed
    1. Qian J, Li M, Zhang X, Wang Q, Zhao J, Tian Z, et al. . Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study. Eur Respir J. (2019) 53:1800081. 10.1183/13993003.00081-2018 - DOI - PubMed
    1. McGoon MD, Krichman A, Farber HW, Barst RJ, Raskob GE, Liou TG, et al. . Design of the REVEAL Registry for US patients with pulmonary arterial hypertension. Mayo Clinic Proc. (2008) 83:923–31. 10.1016/S0025-6196(11)60769-6 - DOI - PubMed
    1. Benza RL, Gomberg-Maitland M, Miller DP, Frost A, Frantz RP, Foreman AJ, et al. . The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. (2012) 141:354–62. 10.1378/chest.11-0676 - DOI - PubMed