Implications of systolic pulmonary artery pressure trajectories in systemic lupus erythematosus-associated pulmonary hypertension: a CSTAR-PAH cohort study.

Authors

Qifang Guo, Dunwu Wu, Xiuling Zhang, Le Yu, Jingjing Shang, Xueqin Feng, Rongwei Zhang, Shaoyuan Mao, Wei Zhou, Xinwang Duan

Published in

Frontiers in immunology. Volume 17. Pages 1819805. Epub Jun 09, 2026.

Abstract

To identify the evolution trajectory of systolic pulmonary artery pressure (sPAP) in patients with systemic lupus erythematosus-related pulmonary arterial hypertension (SLE-PAH) and to evaluate its prognostic significance.
In this study, 81 SLE-PAH patients from the Chinese SLE Treatment and Research Group-Pulmonary Arterial Hypertension (CSTAR-PAH) cohort of the Second Affiliated Hospital of Nanchang University were included for a retrospective cohort study. A group-based trajectory model was used to identify the trajectories of sPAP over time. Baseline factors related to trajectory assignment were analyzed through multiple logistic regression. Univariate and multivariate Cox regression and Kaplan-Meier analysis were used to evaluate the prognostic value of trajectory groups for all-cause mortality, and to analyze the related influencing factors of all-cause mortality.
Four distinct sPAP trajectories were identified: trajectory 1 (n = 17, 20.9%; high initial sPAP with rapid decrease), trajectory 2 (n = 44, 54.3%; moderate initial sPAP with slow decrease), trajectory 3 (n = 13, 16.0%; low initial sPAP with slow decrease), and trajectory 4 (n = 7, 8.6%; high initial sPAP with rapid increase). Longer 6-minute walking distance (6MWD) was independently associated with trajectories 2 (OR = 3.077, p < 0.001) and 3 (OR = 11.106, p < 0.001), whereas shorter 6MWD (OR = 0.220, p < 0.001), elevated B-type natriuretic peptide (OR = 2.159, p < 0.001), higher IL-1β (OR = 1.412, p < 0.001), higher TNF-α (OR = 1.751, p < 0.001), and higher mean pulmonary artery pressure (OR = 1.067, p < 0.001) independently predicted trajectory 4 membership. During follow-up, trajectory 4 remained a strong independent predictor of mortality (HR = 8.843, p = 0.037) after multivariable adjustment, together with higher systemic lupus erythematosus disease activity index (HR = 1.502, p = 0.032), elevated IL-6 (HR = 1.031, p=0.029), higher systemic inflammation response index (HR = 1.828, p = 0.019), and shorter 6MWD (HR = 0.795, p = 0.028). Patients in trajectory 4 exhibited significantly worse survival (log-rank p < 0.0001).
sPAP trajectories capture clinically meaningful heterogeneity in SLE-PAH, with the rapid-progression trajectory identifying a high-risk phenotype characterized by pronounced inflammation, right ventricular strain, and functional compromise. Trajectory-based phenotyping enhances risk stratification and may guide personalized management in SLE-PAH.

PMID:
42344918
Bibliographic data and abstract were imported from PubMed on 25 Jun 2026.

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