September is Pulmonary Fibrosis Awareness Month. Pulmonary fibrosis (PF) is an interstitial lung disease characterized as progressive, irreversible scarring of the lungs, which thickens the lung tissue leading to difficulty breathing and persistent cough.

The most commonly diagnosed form of pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF). This means that no known cause is attributed to the diagnosis, though certain factors such as smoking, older age or family history can increase the risk. There are about 218,000 people in the U.S., with 61,000 estimated new cases in 2020. Currently, there is no cure for PF and pharmacological treatment options are limited to slowing the progression of scarring with the use of drugs called anti-fibrotic agents. Other treatments include oxygen therapy and pulmonary rehab. Doctors may additionally prescribe drugs to help with other symptoms, such as acid reflux, cough and inflammation. In the most severe cases, a lung transplant may be needed.

The limited treatment options for PF means that understanding the basic biology and progression of pulmonary fibrosis are key parts of the puzzle to developing new therapies. Here, we highlight three Lung Association funded researchers and their innovative approaches to improving treatment and prevention of pulmonary fibrosis.

Nunzia Caporarello, Ph.D., is studying the role of the blood vessels of the lung in the development of pulmonary fibrosis at the Mayo Clinic. She was awarded the Lung Association’s Dalsemer Research Grant, a mentored award for early career researchers in the field of interstitial lung disease. Her lab is interested in a protein called ERG, which binds to specific sequences of DNA to influence nearby genes. ERG controls the function and biology of the blood vessels of the lung, but it is not well-known what role blood vessels play in the development of pulmonary fibrosis.

Dr. Caporarello’s recently published paper in Nature Communications discovered that ERG is critical to blood vessel function in the lung, but ERG becomes dysfunctional with aging, resulting in fibrosis. This landmark finding identifies a potential target for treating idiopathic pulmonary fibrosis. Also, this study helps us understand the aging process, a great example of how Lung Association-funded research can lead to additional findings outside of the original idea.

Dr. Edy Kim, M.D., Ph.D., at Brigham and Women’s Hospital in Boston is focusing on a less commonly studied type of lung scarring called unclassifiable lung fibrosis (uILD). It is not currently known what causes uILD, so we lack targeted treatments. Dr. Kim’s lab uses innovative methods which can look at thousands of genes in thousands of cells at the same time, and they found that early uILD, a molecule called CXCL12 recruits immune (T cells) to the lung, which drives inflammation of connective tissue. Understanding these mechanisms may lead to the first targeted treatment for uILD.

Ruogang Zhao, Ph.D., at the University of Buffalo is studying how the immune system is involved in the development of lung fibrosis. Dr. Zhao’s lab developed an experimental model to show how macrophages, a type of immune cell which usually functions by eliminating foreign substances, contribute to the progression of lung fibrosis. They are also examining other factors such as blood flow, tissue stiffness and how different types of macrophages may affect fibrosis formation. This research is important because it helps to improve our understanding of how scar tissue forms in the lung and could lead to the discovery of anti-fibrosis therapies.

Together, these Lung Association researchers are working to determine the various causes of pulmonary fibrosis, using varied approaches in an innovative way and contribute to the collective knowledge generated by researchers around the world.

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