Proteins in Plasma Could Provide Information about IPF Disease Severity

A study of proteins in plasma taken from patients in the IPF-PRO registry found that patients with IPF have a distinct circulating set of proteins and that select proteins correlate well with clinical measures of disease severity.

Certain proteins may provide important information about clinical measures of disease severity in patients with idiopathic pulmonary fibrosis (IPF), according to recent research from the DCRI.

IPF is a devastating lung disease characterized by progressive scarring of lung tissue. Although the incidence and prevalence of IPF are increasing, its clinical diagnosis and management remain challenging. The DCRI oversees the IPF-PRO registry, which has enrolled over 1,000 patients with IPF. The DCRI’s Scott Palmer, MD, MHS, serves as the principal investigator for the registry. The prospective, serially collected clinical data and biosamples in IPF-PRO provide a unique and rich infrastructure from which to grow both clinical and translational research initiatives aimed at improving care for patients with IPF.

The most recent study from the IPF-PRO Registry, which was led by DCRI’s Jamie Todd, MD, MHS, and was published in Respiratory Research, included 300 patients in the registry and 100 patients without known lung disease as a control group. The patients without lung disease were drawn from the MURDOCK study, which is led by Kristin Newby, MD. Plasma was collected from patients at enrollment and analyzed using aptamer-based proteomics. Of more than 1,300 proteins assayed, 551 had statistically significantly different expression in patients with IPF as compared with individuals without lung disease—including 47 proteins that appeared to be at least 1.5 fold different in IPF relative to the control group. Notably, several of these proteins also correlated well with clinical measures of IPF severity, including lung function parameters and indicators of the extent of lung fibrosis observed on CT scans of the lungs.

“This research is the first phase of a stepwise approach,” Todd said. “Now that we have a better understanding of protein expression profiles in these patients and their relationship to the clinical measures we use to assess disease severity, we can conduct further studies to consider the value of these proteins as predictors of disease behavior or patient prognosis. We are really just at the tip of the iceberg of what is possible using the rich repository that is IPF-PRO.”

Todd added that she and her team, including Megan Neely, PhD, Robert (AJ) Overton, and Hillary Mulder of DCRI Biostatistics, are now embarking on this effort. Specifically, the team is using multivariable modeling approaches to consider whether sets of these proteins can provide information about clinically relevant outcomes in patients with IPF.

“Early observations indicate that some proteins confer important information about future outcomes in patients with IPF—information that has not been completely captured by considering clinical factors alone,” Todd said. Her team has submitted an abstract with these early observations to the American Thoracic Society meeting.