The following limitations exist in this study: (1) increasing the sample size and improving the genetic information may improve the accuracy of disease assessment and prediction; (2) the expression of rabep1 should be detected by overexpressing or knocking down JPX in cell lines, and then exploring the molecular mechanism; (3) the PAH model in mice cannot fully imitate the human PAH process, and human lung and heart clinical samples need further study to verify the relationship between immune-related lncRNA-mRNA pairs and PAH. Here, RABEP1 is linked to pulmonary arterial hypertension.