3 identified clusters of pulmonary fibrosis patients | MedNet

An international study found 3 groups of patients with pulmonary fibrosis based on blood biomarkers. According to the researchers, this provides an opportunity to develop targeted therapy.

The aim of the study was to classify patients with pulmonary fibrosis by blood biomarkers for different disease patterns (endotypes). The researchers classified 455 (348 men, 107 women; mean age 72 years) of 580 patients from the PROFILE study: a multicenter, prospective cohort of individuals with idiopathic pulmonary fibrosis or interstitial pneumonia or -specialized in 2 English hospitals. In the PROFILE study, 13 blood biomarkers for extracellular matrix change, epithelial stress and thrombosis were measured using ELISA. Patient cohort was assessed with a biomarker machine learning program applied to the Australian Idiopathic Pulmonary Fibrosis Registry (AIPFR) database.

Three clusters were identified in the analyzed group. There was a high accumulation of PRO-C4, PRO-C28, C3M and C6M in the BM collection (‘basement membrane’; n = 248). There was a high concentration of MMP-7, SP-D, CYFRA211, CA19-9 and CA-125 in the EI cluster (‘epithelial lesion’; n = 109). There was a high concentration of X-FIB in the 3rd group (XF: ‘fibrin crosslinked’; n = 98). The same 3 groups were found in the AIPFR database. In the PROFILE database, the EI and XF clusters were associated with a higher likelihood of mortality than the BM cluster. The EI cluster showed the highest annual decrease in percent FVC, followed by the BM and the XF cluster. This pattern was also seen in the AIPFR database.

Based on blood biomarkers, 3 groups of pulmonary fibrosis patients can be identified that are related to lung function and prognosis. This shows that there are endotypes of pulmonary fibrosis, with the potential to develop targeted therapy.

Source:

Fainberg HP, Moodley Y, Triguero I, et al. Cluster analysis of blood biomarkers to identify molecular patterns in pulmonary fibrosis: a multicenter, prospective, observational cohort evaluation with independent validation. Lancet Respir Med. 2024; 12:681-92.