New study aims to find early drivers of pulmonary fibrosis in at-risk patient population

Reviewed by Emily Henderson, B.Sc.Oct 6 2021

Researchers at Vanderbilt University Medical Center are leading a study designed to identify early drivers of pulmonary fibrosis in an at-risk patient population. Funding for the research, which could help predict early disease at the cellular level and possibly drive the development of novel therapies, is being provided by Three Lakes Foundation.

Pulmonary fibrosis (PF) is a rare disease that causes scarring of the lung tissue. Each year, 40,000-50,000 individuals are diagnosed with PF in the US, and 40,000 lose their lives to the disease annually. Currently, there are no available therapies for PF.

To develop transformative therapies, it is essential to understand what initiates the disease and what drives progression of the disease. To do this, research must focus on the very earliest aspect of the disease – before people develop symptoms and before it can be detected on X-rays or CT scans of the lung."

Jonathan Kropski, MD, Assistant Professor, Department of Medicine, Vanderbilt University Medical Center

Dr. Kropski will oversee study operations, analysis and coordination among the sites along with Nicholas Banovich, Ph.D., an associate professor at the Translational Genomics Research Institute (TGen), an affiliate of City of Hope and a leading genomics institute. Dr. Banovich, who is an expert in human genetics, genomics and single-cell biology, will oversee analysis of lung tissue samples.

The research team will use a state-of-the-art platform to sequence single-cell tissue collected from individuals with a family history of pulmonary fibrosis. This unique lung tissue contains biospecimens from pre-symptomatic individuals and offers unprecedented opportunity to investigate the beginning of early interstitial lung disease.

"This study expands our work in established disease," said Cheryl Nickerson-Nutter, Ph.D., VP of research and development at Three Lakes Foundation. "We believe it will provide critical clues in how PF develops and ultimately lead to discovery of new therapies capable of stopping and reversing disease progression."