A Study From Yale University is Helping Solve The Mystery of Idiopathic Pulmonary Fibrosis

According to a story from seas.yale.edu, a recent study conducted under the supervision of Yale University is providing new clues for the underlying cause of idiopathic pulmonary fibrosis. The researchwea observed how the impact of cell behavior can contribute to creating the conditions for the disease to begin.

Idiopathic pulmonary fibrosis (IPF) refers to pulmonary fibrosis in which the cause is unknown. The disease is characterized by progressively worsening scarring of the lungs, which causes a steady decline in respiratory function. Smoking cigarettes, a family history of the disease, and certain viral infections are risk factors for the condition.
While there are medications that can help slow the effects of the disease, average live expectancy post diagnosis is only four years. A lung transplant may be possible for some people with the condition. Some symptoms include shortness of breath and a dry cough, as well as fatigue and clubbing of the digits. These symptoms tend to develop slowly. Some patients experience acute exacerbation, in which symptoms suddenly worsen dramatically; this decline is often fatal. To learn more about idiopathic pulmonary fibrosis, click here.

The research focused on the role that a specific type of cell plays in the development of idiopathic pulmonary fibrosis. The cell in question is called a pericyte. These cells surround many of the smaller blood vessels in the lungs, otherwise known as microvessels. Despite their small size, these cells are essential for the function of the lungs, heart, and brain. Microvessels are often destroyed as IPF begins to worsen. Experts originally assumed that the pericyte cells also died, but upon closer study, it was clear that pericytes were present at the sites of tissue scarring. The survival of the pericytes also means that the microvessels are still present as well, but they are unable to function.

Meanwhile, the pericytes begin to secrete a protein that is responsible for the hardening and stiffening of tissue that occurs and forms scarring. This process begins after the cells are exposed to certain factors and changes in their environment. However, after treating the cells with a drug, they were able to reverse this process in a human lung model, and the lung tissues became soft again. This could lead to the development of new treatments for IPF.


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