Is Pulmonary Fibrosis Genetic?

Although most cases of pulmonary fibrosis are not genetic and often unknown when it is called idiopathic pulmonary fibrosis. However, there are rare cases where the condition runs in families suggesting a genetic link to the disease.

MORE: The relationship between pulmonary fibrosis and pulmonary hypertension.

Researchers have isolated two genes that may be responsible for developing the condition: TERT and TERC. In around 15 percent of IPF cases, there are mutations in these two genes, although researchers have not yet established exactly why this happens.

In familial pulmonary fibrosis, one copy of the mutated gene in each cell could be enough for the condition to develop, but not everyone who has the mutated cell goes on to develop the disease, meaning that it is a sporadically inherited disease.

The disease does not necessarily pass down from one generation to the next, in some cases of genetic IPF there are no known cases of it within the family. Read more about genetic pulmonary fibrosis here.

MORE: One family’s battle with pulmonary fibrosis.

Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.


  1. Lesley brown says:

    I’m a cystic fibrosis carrier has there been any scientific enquiry into there being a connection between the two fibrosis. I know the diseases are different but it’s a question ?

  2. Chrissy Stirland says:

    My brother has been diagnosed with IPF and I found out yesterday that my mother’s cousin has also got the same condition. As this condition is relatively rare is this likely to be genetic?

    • Jennifer Pardue says:

      I just lost my amazing father to this rare and sneaky disease, just one months ago. It was the hardest thing to see there towards the end… He fought so hard! And was actually doing pretty good, and even walking again. Then about a week later, he was gone. He was on hospice and I know their job is to “keep him comfortable”. But it’s still not fair! He has so much life left in him.
      So I wonder the same thing about genetics. And if there is a test that can be taken, To find out if my sister and I are at risk.

      Many blessings to all patients and families going through this. 🙏🏻

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