Circulating CTHRC1 levels are associated with IPF disease severity and survival

Area of Science:

• Pulmonary Medicine
• Biomarker Discovery
• Fibrotic Lung Diseases

Background:

• Idiopathic pulmonary fibrosis (IPF) is a severe lung disease with high mortality.
• A novel subset of pathological fibroblasts expressing CTHRC1 is uniquely found in fibrotic lung diseases.

Purpose of the Study:

• To investigate the association between serum CTHRC1 levels and clinical outcomes in IPF patients.
• To evaluate CTHRC1 as a potential biologically relevant biomarker for IPF.

Main Methods:

• Retrospective longitudinal study of two IPF cohorts (UCSF, PFF) and healthy controls.
• Serum CTHRC1 measured by ELISA; patients stratified by CTHRC1 quartiles.
• Analysis of associations between CTHRC1 and lung function, lung function trajectory, and transplant-free survival.

Main Results:

• Serum CTHRC1 was significantly elevated in IPF patients compared to healthy controls.
• Higher CTHRC1 levels correlated with lower baseline lung function (FVC, DLCO) and accelerated FVC decline.
• Elevated CTHRC1 was associated with worse transplant-free survival in both cohorts.

Conclusions:

• Circulating CTHRC1 is elevated in IPF and linked to increased disease severity.
• CTHRC1 may serve as a valuable biomarker reflecting the burden of pathological fibroblasts in IPF.
• These findings underscore the biological significance of CTHRC1 in IPF pathogenesis and prognosis.