Clinical characteristics and prognosis in systemic lupus erythematosus-associated pulmonary arterial hypertension based on consensus clustering and risk prediction model

Abstract

Abstract Background: This study aims to probe the clinical characteristics and prognosis in SLE-PAH based on consensus clustering and risk prediction model. Methods: A total of 163 SLE-PAH patients were enrolled retrospectively based on medical records at the First Affiliated Hospital of Zhengzhou University from July 2014 to June 2021. Unsupervised consensus clustering was used to identify SLE-PAH subtypes that best represent the data pattern. The Kaplan-Meier survival was analyzed in different subtypes. Besides, the least absolute shrinkage and selection operator (LASSO) combining with Cox proportional hazards regression model were performed to construct the SLE-PAH risk prediction model. Results: Clustering analysis defined two subtypes, cluster 1 (n = 134) and cluster 2 (n = 29). Compared with cluster 1, SLE-PAH patients in cluster 2 had less favorable levels of poor cardiac, kidney, and coagulation functions, along with high SLE disease activity, low survival rate within 2 years (86.2% vs. 92.8%) (P < 0.05). The risk prediction model was then constructed, including older age (≥ 38 years), anti-dsDNA antibody, neuropsychiatric lupus and platelet distribution width (PDW). Conclusions: Consensus clustering identified two distinct SLE-PAH subtypes, of which cluster 2had more organ involvement, higher disease activity, and poorer survivability within 2 years. Older age (≥38 years), anti-dsDNA antibody, neuropsychiatric lupus and PDW could be regarded as prognostic factors for death with SLE-PAH patients.