Precision Medicine Research for Pulmonary Fibrosis May Bring New Treatments Closer

Personalized pulmonary fibrosis treatment another step closer to reality as new study techniques and improved funding advance research

Hospitals and health systems may soon gain new treatment options for idiopathic pulmonary fibrosis (IPF), a chronic, progressive lung disease that is ultimately fatal. IPF affects over 200,000 Americans, and the fact that IPF is poorly understood, as well as difficult and costly to treat, makes it ideal for precision medicine research and research grant support.

Well underway, for example, is the PRECISIONS (Prospective tReatment EffiCacy in IPF uSIng genOtype for Nac Selection) study. The study is made possible by a recent $22 million grant from the National Institutes of Health (NIH) and Three Lakes Partners, a philanthropic family organization. Researchers want to improve both precision medicine diagnostics and treatments to improve the outcomes of IPF patients and enable hospitals the ability to provide better care for this disease.

PRECISIONS uses data from patients enrolled in the Pulmonary Fibrosis Foundation’s (PFF’s) Patient Registry and Biorepository. The PFF has 42 care centers across the United States and has enrolled over 2,000 patients with a range of different types of pulmonary fibrosis disorders.

Interstitial Lung Diseases Focus of Precision Medicine Research

Fernando Martinez, MD, MS (above), and others part of the PRECISIONS study are working to advance personalized treatments for idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases. (Photo: Weill Cornell Medicine)

“This innovative study highlights the value of a partnership between a broad range of investigators, the PFF, a philanthropic organization (Three Lakes Partners), and the National Heart, Lung, and Blood Institute (NHLBI),” explained lead researcher Fernando Martinez, MD, MS, in a statement by Weill Cornell Medicine, where pulmonologists such as Martinez and others are developing a new paradigm to confront IPF and other forms of progressive fibrotic interstitial lung diseases, including chronic hypersensitivity pneumonitis (CHP). “Most importantly, it [PRECISIONS] seeks to provide patients with interstitial lung disease (ILD) access to personalized medicine in which the right medication is used for the right patient.”

Said Imre Noth, MD, co-principal investigator, in a PFF statement, “PRECISIONS has the potential to really change the scientific landscape over how we view IPF and ILDs by providing molecular classifications while determining if a pharmacogenetically driven treatment can change outcomes.”

The grant funding the PRECISIONS study will be a significant boost to precision medicine for pulmonary fibrosis, but it is not the only recent advancement in this area.

IPF Treatments Tested Using New Methods

A separate IPF study published in APL Bioengineering in November 2021 and part of “A scalable 3D tissue culture pipeline to enable functional therapeutic screening for pulmonary fibrosis” described a newly developed method of growing lung cells that allows researchers to perform more in-depth study of pulmonary fibrosis outside of the body. The researchers from University of Minnesota-Twin Cities and Mayo Clinic found a new method of growing lung cells in 3D forms. This new method appears simple to replicate when compared to other similar studies.

“Many organoid and lab-on-a-chip platforms can be hard to use,” said David Wood, PhD, an associate professor at the University of Minnesota Department of Biomedical Engineering and co-author of the PF functional therapeutic screening study. “What’s exciting is that this system is very easy to use. We’ve already disseminated it to two other labs who are using it completely independent of us.”

This new method of growing lung cells mimicking the lungs of patients with IPF will allow researchers the ability to test multiple different treatment combinations that would be impossible to attempt in a single patient. It also allows the researchers and, in the future, clinicians to grow cells from specific patients with different genetic profiles, allowing for precision medicine treatments for different forms of pulmonary fibrosis.

New Lab Tools and Models Contributing to Outcomes Research

“IPF is a horrible disease that drastically impacts a patient’s life and eventually causes them to die from lack of oxygen,” said Katherine Cummins, co-author and PhD candidate at the University of Minnesota Department of Biomedical Engineering. “It’s really important to have lab tools and models that create and control the microenvironment in which the cells sit, because this may be key to preclinical identification of possible treatments.”

While idiopathic pulmonary fibrosis is still a difficult to treat disease that is ultimately fatal, hospitals may soon see new precision medicine treatment options for these patients as research in this field begins to flourish. IPF research using precision medicine is still in its early days, but forward-looking hospital and pulmonology leaders will begin paying attention now to the developments that are likely to emerge in precision medicine pulmonary fibrosis treatments.

—Caleb Williams

Related Information:

Three Lakes Partners

Dr. Martinez Leads PRECISIONS Study on Idiopathic Pulmonary Fibrosis (IPF)

NIH Awards Major Grant in Pulmonary Fibrosis Research

Scalable 3D Tissue Culture Pipeline to Enable Functional Therapeutic Screening for Pulmonary Fibrosis

David Wood, PhD

Katherine Cummins

Fernando Martinez, MD, MS

Imre Noth, MD

Interviews With Precision Medicine Movers