Abstract

<b>Background:</b> Idiopathic pulmonary fibrosis (IPF) is a highly fatal lung disease with a median survival of 2-4 yeas. The poor prognosis is due to the limited therapies on the one hand, and the lack of effective prognostic indicators on the other hand. This study&nbsp;aimed to construct a novel prognostic signature of IPF to assist the management of IPF patients in the process of treatment. <b>Methods:</b> IPF samples were acquired from&nbsp;Gene Expression Omnibus(GEO). immune-related genes (IRGs)&nbsp;were obtained from ImmProt database. Univariate Cox regression analysis was adopted to screen significantly prognostic IRGs for IPF patients. Multiple Cox regression analysis was used to construct the prognostic signature. <b>Results:</b> A total of 112 IPF patients were included in this study. Compared with healthy subjects, there were a total of 52 prognosis-related DEGs of IPF patients, of which 37 genes were identified as IRGs. Five genes (CXCL14, SLC40A1, RNASE3, CCR3, and RORA) out of these IRGs were significantly associated with overall survival (OS) and utilized for establishment of the prognostic signature. IPF patients were divided into high-risk and low-risk groups based on the prognostic signature. Significant differences in the OS probability were observed between high-risk and low-risk IPF patients in both training cohort and validation cohort. The AUCs of the ROC curve for the prognostic signature in the training cohort and validation cohort were 0.858 and 0.837, respectively. <b>Conclusion:</b> In conclusion, we developed a validated and reproducible IRG-based prognostic signature that should be helpful for the personalized management of patients with IPF.

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