High turnover of types III, VI collagen predicts mortality in progressive IPF

High turnover of blood biomarkers of types III and VI collagen is predictive of disease activity, short-term progression, and long-term mortality in patients with progressive idiopathic pulmonary fibrosis (IPF), a recent study has shown.

“These biomarkers can help to identify patients with a high extracellular matrix (ECM) remodeling phenotype at high risk of disease progression and death,” the investigators said.

One hundred eighty-five patients with newly diagnosed IPF were enrolled and had their neoepitope biomarkers of types III and VI collagen turnover (C3M, C6M, PRO-C3, and PRO-C6) measured. Lung function tests and 6-minute walk tests were used to assess disease severity at baseline and progression over 6 months. The investigators then examined all-cause mortality over a 3-year follow-up period.

High baseline levels of C3M, C6M, PRO-C3, and PRO-C6 correlated with more advanced disease at the time of diagnosis, while baseline levels of C6M (hazard ratio [HR], 2.3, 95 percent confidence interval [CI], 1.3–3.9) and PRO-C3 (HR, 1.8, 95 percent CI, 1.1–3.0; p=0.03) correlated with mortality over 3 years of follow-up.

Patients with several increased biomarkers at baseline, indicative of a high ECM remodeling phenotype, had more advanced disease at baseline, higher risk of progression or death at 6 months (odds ratio, 1.4, 95 percent CI, 1.1–1.8; p=0.002), and higher mortality over 3 years of follow-up (HR, 2.4, 95 percent CI, 1.3–4.5; p=0.007).

“Prediction of IPF progression is vital for the choice and timing of treatment and patient follow-up,” the investigators said. “This could potentially be achieved by prognostic blood biomarkers of ECM remodeling.”