The decreased expression of 4 genes—CD28, ICOS, LCK, and ITK—predicted shorter survival time in patients with idiopathic pulmonary fibrosis (IPF), according to the results of a study led by Yale University researchers. The team, made up of researchers from Yale, University of Chicago, and University of Pittsburgh, believes the gene set should be further evaluated for use in patient prioritization for lung transplantation and stratification in drug studies.

“Given the fact that lung transplantation is the only therapy that has shown to improve survival in IPF, our test could allow physicians to refer IPF patients for lung transplant at the right time – not too late and not too early,” said senior and corresponding author Naftali Kaminski, MD, professor and chief of pulmonary, critical care, and sleep medicine at Yale School of Medicine.

Using two cohorts of patients, investigators analyzed the expression of the genes in the whole genome of patients with IPF, and identified 52 genes that significantly correlated with outcome. They found that decreased numbers of CD28, ICOS, LCK, and ITK not only predicted shorter survival time, but they were mostly related to immune activation.

“Our result may also shed light on disease mechanisms, by supporting the emerging notion that aberrant immunity may play a role in IPF,” said Imre Noth, MD, associate professor of Medicine, University of Chicago Hospitals and Clinics and leader of the University of Chicago team.