Pulmonary fibrosis is a disease marked by scarring in the lungs. Tissue deep in the lungs becomes thick, stiff and scarred. The scarring is called fibrosis. As the lung tissue becomes scarred, it interferes with a person’s ability to breathe. In some cases, the cause of pulmonary fibrosis can be found. But most cases of pulmonary fibrosis have no known cause. These cases are called idiopathic pulmonary fibrosis (IPF).
Some research has advanced our understanding of IPF, yet there is a desperate need for more. For example, we know that a person’s risk for IPF is higher in people who smoke or have a family history of IPF, and the risk increases with age. The most common symptoms of IPF are shortness of breath and cough. Some people may not have symptoms at first, but symptoms can develop and get worse as the disease progresses.
The way that IPF progresses varies from person to person, and scarring may happen slowly or quickly. In some people, the disease stays the same for years. In other people, the condition quickly gets worse. Many people with IPF also experience what are known as acute exacerbations, where symptoms suddenly become much more serious. Other complications of IPF may include pulmonary hypertension or respiratory failure, which happen when the lungs cannot deliver enough oxygen into the bloodstream without support. This prevents the brain and other organs from getting the oxygen they need.
Currently, there is no cure. People with severe and rapidly worsening IPF live only about three to five years after diagnosis. Current treatments for pulmonary fibrosis slow down the disease but cannot remove scarring that has already occurred. That is why there is an urgent need for research that will lead to treatments that can stop and even reverse scarring in the lungs of people with pulmonary fibrosis. The Lung Association Research Institute is funding research which will advance our knowledge of this disease.
One example is Lung Association grant recipient Tatsuya Tsukui, Ph.D., Assistant Professor of Pulmonary, Critical Care, Allergy and Sleep Medicine at the University of California, San Francisco, who hopes his research will contribute to life-saving new treatments for people with pulmonary fibrosis. His research focuses on inflammation and cells called fibroblasts.
How Pulmonary Fibrosis Develops
Lungs affected by pulmonary fibrosis are chronically inflamed. Inflammation is a typical reaction to tissue injury in the lung. It aims to remove harmful organisms and start the healing process to restore lung function. When inflammation becomes chronic, it causes ongoing tissue damage and fibrosis. Fibroblasts are cells in the tissue that are among the first to respond to injury by detecting damage and starting inflammation. In lungs affected by IPF, abnormal fibroblasts gather at injury sites and produce too much of the protein, collagen. This buildup of collagen leads to the stiff fibrotic tissue that interferes with the activity of the lung.
Grant Funding to focus on Inflammation and Fibroblasts
Dr. Tsukui, Assistant Professor of Pulmonary, Critical Care, Allergy and Sleep Medicine at the University of California, San Francisco, recently identified subgroups of fibroblasts that are stimulated by distinct chemical signals, leading to inflammation in pulmonary fibrosis. “We don’t yet understand the impact of these diverse inflammatory signals on the behavior of diseased fibroblasts,” he said.
With an American Lung Association Hastings Innovation Award for Interstitial Lung Disease, Dr. Tsukui aims to understand the role of these inflammatory signals on fibroblasts in pulmonary fibrosis. Using technology called single-cell RNA sequencing, he has discovered new subtypes of fibroblasts with unique inflammatory signal patterns in pulmonary fibrosis. He has developed innovative tools that are capable of targeting specific subgroups of fibroblasts. Using these tools, he will study the functions of fibroblast-specific inflammatory signals in mouse models of lung fibrosis.
“By targeting fibroblasts, we hope to stop collagen accumulation in fibrotic lungs,” he said. “Our research will shed light on the understudied role of inflammatory signaling in diseased fibroblasts. Our hope is this will lead to potential novel therapeutic strategies targeting inflammatory fibroblasts in pulmonary fibrosis. This could be life-changing for people with the disease, helping them to breathe well once again.”
To learn more about the groundbreaking research performed by Lung Association researchers, visit lung.org/research.
Blog last updated: October 1, 2024
