A Recent Study from Yale Tracks the Progression of Scarring in Idiopathic Pulmonary Fibrosis

According to a story from EurekAlert!, a collaborative study headed by researchers from Yale University has furthered the understanding of the mechanisms and progression of fibrosis (scarring) in patients with the rare lung disease idiopathic pulmonary fibrosis. The findings from the research will be useful for the development of future therapies with the goal of slowing or halting the progression process.

About Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis is a deadly, chronic, progressive lung disease which is characterized by lung tissue scarring, leading to a decline in lung function over time. The cause of idiopathic pulmonary fibrosis is unknown. With that being said, there are a few risk factors that have been identified, such as smoking cigarettes, exposure to various dusts (metal, wood, stone, and coal dust), occupations related to farming, family history, and potentially certain viral infections. Symptoms include shortness of breath, a dry cough, a distinctive crackling sound detected with a stethoscope, oxygen deficiency in the blood, and clubbed digits. There are few treatment options that can have a significant impact on the progression of idiopathic pulmonary fibrosis. Treatment may include certain medications, pulmonary rehabilitation, oxygen therapy, and lung transplant. Early intervention can make a major difference in outcomes; five year survival rate is between 20 and 40 percent. To learn more about idiopathic pulmonary fibrosis, click here.

Study Results

The initial finding from the study was the observation that lung tissue samples from patients with the disease revealed that even areas of lung the appeared to be unaffected were already subjected to a pattern of genetic alterations. With this knowledge, the scientists tracked the changes to the affected genes as the lung tissue began to scar.

The researchers tracked the genetic changes using RNA sequencing and microRNA measurements. The three most significant findings from the study can be summarized as follows:

  1. The realization that lung tissue that appeared to be health was in fact abnormal and was undergoing genetic changes.
  2. The identification of changes to gene expression that could be distinctive to tissue in various stages of fibrosis.
  3. The identification of molecular regulators that were linked to different stages of fibrosis (early, progressive, and end-stage).

The original study was published in the journal JCI Insight. Check it out here.

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