According to investigators at the University of Pittsburgh School of Medicine and Centocor R&D, a group of blood proteins can foresee which patients with idiopathic pulmonary fibrosis (IPF) – a progressive lung disease – are more likely to die within two years or live at least five years. The discoveries could assist doctors in deciding which patients require a lung transplantation urgently from those who can wait longer. The findings were published online last week in the American Journal of Respiratory and Critical Care Medicine.

IPF causes scarring or thickening of the lungs making breathing increasingly difficult. 50% of individuals with IPF die within three years of diagnosis, but other individuals will do well for long periods of time, said researcher Naftali Kaminski, M.D., professor of medicine, pathology, human genetics and computational biology, Pitt School of Medicine, and director, The Dorothy P. & Richard P. Simmons Center for Interstitial Lung Disease at UPMC.

Dr. Kaminski explained:

“It’s hard to tell based on symptoms alone which patients are in the greatest danger. This biomarker panel has predictive power that can guide our treatment plan. It may also help us design more effective research trials because we’ll be able to better match experimental therapies with the most appropriate patients.”

Blood samples were taken from 241 individuals with IPF. The researchers then measured the levels of 92 candidate proteins in 140 patients and discovered that poor survival, transplant-free survival as well as progression-free survival regardless of sex, age and baseline pulmonary function, were predicted by higher concentrations of five specific proteins that are produced due to the breakdown of lung tissue.

Based on both groups, the team developed the personal clinical and molecular mortality prediction index (PCMI) which includes lung functions, sex as well as levels of one protein, called MMP7, in the blood. They discovered that individuals with a low PCMI were most likely to live more than five years, however, for individuals with a high PCMI the median survival rate was 1.5 years.

Lead author Thomas Richards, Ph.D., assistant professor of medicine and head of the Simmons Center biostatistics team, said:

“This indicates that these blood biomarker levels are not just a reflection of current severity of the lung disease, but they are predictive of impending death.”

Senior author Kevin Gibson, M.D., medical director of the Simmons Center, explained:

“They have the potential to greatly improve our treatment strategies for IPF, in part by showing us which patients have the most urgent need for lung transplant, which is currently the only cure for the disease.”

Mark T. Gladwin, M.D., chief, Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh School of Medicine, stated:

“These findings provide proof of the concept of personalized medicine. We can use a combination of biological and clinical markers to determine the very best care for each patient.”

Drs. Kaminski, Gibson and team have investigations underway in order to get a clear idea on how the biomarkers change over time.

The team includes Kathleen O. Lindell, Ph.D., R.N., and others at the Simmons Center and Pitt School of Medicine, as well as investigators from Centocor R & D in Radnor, Pa.

Written by Grace Rattue