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.

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  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. 🙏🏻

      • I was diagnosed recently as having a genetic case of pulmonary fibrosis. I am so terribly sorry for your loss. I understand what you expressed because I lost my father to Mesothelioma and watching the progression of his lung disease was brutally sad for me. Now, I have a good idea of where this ILD will eventually take me, provided the good Lord doesn’t see fit to take me some other way. Sending blessings to you in your grieving, I know it’s the hardest thing a daughter ever will face losing her Dad. ✝️

  3. My father died of Fibro Alvelotis and I now l believe there is a genetic link with Heamochromatosis . This is in that side of the family. It is interesting abut the oxygen. If there was a starvation of oxygen in the blood could that not cause a minor bruise on the brain resulting in epilepsy??

  4. Lana Bdeir says:

    My father passed away ten years ago from IPF at age 62. His older sister who never smoked a thing in her life died from it 5 years later, and recently his two other sisters (he has 7 sisters), were diagnosed with PF. They caught it early and have been controlling it with meds but I am very concerned about it transferring down to the rest of the family. Does the above mean us kids (next generation) are vulnerable to getting it? I can’t seem to find conclusive info about that online.
    Also, for those who have it, is there a new cure for it?
    Appreciate any info you guys know about it.

  5. David Pecoraro says:

    My grandmother died of IPF, her daughter/my mother died of IPF and I was diagnosed with IPF at 50 and had a lung transplant at 53
    I will be 4 years out 8/6.

  6. Ann Dunne says:

    I don’t have Pulmonary Fibrosis Disease but I do have Chronic Obstructive Pulmonary Disease, I was diagnosed after a bout of Pneumonia back in 2015. I had a huge issue with been out of breath because I was overweight but I joined slimming world & lost 4stone & I can walk now and have no problem with my breathing. I take two inhalers every day that keeps me well. I was lucky to have been put on a exercise Physio program just after been diagnosed with Brian Fitzgibbon from St Lukes Hospital in Kilkenny in the District Hospital in Carlow for 8 weeks. Then we set up our own COPD Support Group in Carlow Town where we have a professional from Sli Blu in Dublin doing a one hour exercise class every Wednesday from 12 to 1pm

  7. Nicole Carratt says:

    This has to be genetic somehow!! All stories of families having it! My uncle died from it my aunt died from it now my dad is sick with it! Is there some type of test to know if your a carrier? I would like to know for my kids but also for myself!! Does both parents have to be a carrier?

  8. Dr. Jakobsen says:

    I lost my wonderful mother some years back at Christmas time from IPF, something she saw coming for decades. As early as 4 decades earlier, after many x-rays and admissions for bronchitis and pneumonias, doctors already suspected something was wrong. Her lungs bore the telltale signs of fibrosis and she lived in the shadow of this realization for more than half her life. She always lamented that she’d likely have a short life. While sadly, this may be true for some, in the end she lived to the ripe age of 81. On oxygen for comfort, and with the limitations expected of an octogenarian, she was well until several weeks before her passing. Living in the shadow of IPF, she lived a rich life, a life of love, purpose, humor, and meaning. IPF may affect your life but it need not define it.

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