Interstitial lung disease causes harmful scarring in the lungs that can require lung transplants.
UVA Health lung researchers are developing a promising approach to detecting patients at risk of interstitial lung disease (ILD), an increasingly common condition that is a leading reason for lung transplants. The approach could accelerate the development of new and better treatments with more tolerable side effects than existing options.
The UVA scientists have already discovered biological indicators in the blood – “biomarkers” – that can predict the survival chances of patients with ILD. But the researchers, led by John S. Kim, MD, believe these types of biomarkers can be used for far more: The scientists are aiming to determine if biomarkers can identify people who have not yet developed ILD but will go on to do so. They also hope to map out the molecular hallmarks of early and late-stage ILD to better understand the condition and how it develops, as well as to find ways to prevent it.
“Oftentimes when patients are diagnosed with interstitial lung disease, they already have a lot of damage to their lungs that may not recover and limits how effective treatments can be,” said Kim, of UVA Health’s Division of Pulmonary and Critical Care. “Our goal is to identify treatments that can prevent ILD, and an important step to do this is identify adults at risk of developing this disease in the future.”
Understanding Interstitial Lung Disease
ILD is a group of chronic respiratory conditions that cause inflammation and scarring in the lung tissue. The most common type is idiopathic pulmonary fibrosis (IPF), the cause of which is not entirely understood. Some forms of ILD progress faster than others, but some are life-threatening, and there is no cure. Approximately a third of all lung transplants each year go to patients with ILD.
There are drugs available that can slow the progression of IPF, but these drugs can have side effects, such as affecting liver function, that can leave patients unable to take them. Many patients also suffer from gastrointestinal issues such as nausea, vomiting and diarrhea, so new and better treatments are much needed.
Kim and colleagues recently published a research study in the American Journal of Respiratory and Critical Care Medicine that identified several plasma protein biomarkers that were dassociate with new-onset ILD. Notably, some of these proteins were highly expressed in lung tissue of patients with ILD. Kim and his collaborators hope to build off this published work and help doctors better understand ILD and IPF and find new ways to treat the conditions. The researchers have received a five-year grant from the National Institutes of Health’s National Heart, Lung and Blood Institute to take the next steps in their research, which they hope will ultimately allow doctors to enroll people at risk of developing ILD in preventative clinical trials.
“We aim to identify blood biomarkers that predict both the development of ILD and survival in patients with disease,” said Kim, of the UVA School of Medicine’s Department of Medicine. “We hope these biomarkers can complement other tools like lung imaging and genomics to identify adults at higher risk of developing ILD, which will be crucial to developing treatments aimed at preventing this disease.”
Findings Published
Kim and his colleagues have described their biomarker findings in a scientific paper in the American Journal of Respiratory and Critical Care Medicine. The research team consisted of Kim, Catherine L. Debban, Daniel Guzman, Riley T. Hannan, Mary Salvatore, Claire McGroder, David Zhang, Anna J. Podolanczuk, Daniel A. Duprez, Shwu-Fan Ma, Yong Huang, Jeffrey M. Sturek, Stephen S. Rich, Jerome I. Rotter, Rajat Deo, Ruth F Dubin, Janelle Vu Pugashetti, Janelle Vu Pugashetti, Jennifer M Wang, Jennifer M. Wang, MeiLan K. Han, Justin M. Oldham, Imre Noth, Prescott G. Woodruff, Victor E. Ortega, Julie C. Fanburg-Smith, Edward B. Stelow, Christopher A. Moskaluk, Eric A. Hoffman, Eric A. Hoffman, Christine Kim Garcia, Russell P. Bowler, Peter Ganz, R. Graham Barr and Ani Manichaikul.
A full list of funding sources for the research and the Multi-Ethnic Study of Atherosclerosis (MESA) Lung Study and Subpopulations and Intermediate Outcome Measures in COPD (SPIROMICS) the scientists used to conduct the research is included in the paper.
The next steps in the UVA Health research is being funded by NHLBI grant R01HL176659-01A1.
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