Wednesday, August 2, 2017

What’s new in pulmonary fibrosis?

It’s been a while since I posted here. Work & life…

Thought I would give an update on what’s new in the world of pulmonary fibrosis:

1. I posted earlier this week that the PFF is holding its Summit meeting in Nashville in November. All are welcome! go to to learn more and register.

2. Two studies have now suggested that drugs approved in many parts of the world to treat IPF (nintedanib and pirfenidone) may reduce the risk of respiratory problems requiring hospitalization in people living with IPF: and

3. Did you know that current medical guidelines recommend the use of antacids to treat IPF? At the time these guidelines were issued in 2015, this recommend was somewhat controversial, and now newer evidence suggests that antacids are linked to worse outcomes in IPF in some cases. A recent publication in a medical journal describes the issue in great detail:

4. My research group has recently published a paper linking sleep apnea to early stages of interstitial lung disease. This is far from definitive proof that sleep apnea contributes to interstitial lung disease or PF, but it is suggestive enough that we are pursuing more research in this area.

5. For those of you who are putting off exercise, a recent study suggests that exercise in pulmonary rehabilitation benefits people living with interstitial lung disease:

I’ll try to post more frequently as time allows.



  1. Doctor Lederer thank you so much for taking the time to post this. It's great news that OFEV and Esbriet are helping many people. Hoping more studies are being done.
    I have a question on #4. I have friends with sleep apnea can you explain why it may contribute to ILD's.

    1. Hi Laura. We still don't really know whether sleep apnea might contribute to the risk of developing an ILD, or if people with ILD must just be more likely to develop sleep apnea after their ILD begins. I run a research program that includes studying whether sleep apnea is a cause of ILD. We published our first paper very recently. You can read the abstract here:


  2. EXERCISE! I cannot emphasize enough how much good it does! I began regular exercise after going through pulmonary rehab. I also began using oxygen at night. I learned how to breath properly and was able to get enough good sleep at night that getting up to go work out was possible. Now it is necessary - I feel better. I do a cardio workout for 30 minutes, then work with weights to strengthen my abs, sides and back to help my breathing. Just added workout for my knees, gluts and pelvic/thigh girtle muscles to strengthen my legs and hips for walking.
    My advise: do what you can, do it on a regular basis, do it with proper guidance and supervision. (I work out at the same facility that provided the pulmonary rehab.) And breath!

    1. Thanks for sharing your experience, Anne. Completely agree - exercise is a great treatment for many with PF. (talk to your doctor first!)

  3. So much talk about IPF, what about NSIP? Isn't fibrosis damaging the same with both diseases? why medicine for IPF is not prescribed for NSIP? we need to stop the fibrosis, don't we?

    1. I completely agree. There are some studies being performed for people with PF who don't have IPF. Here is a link to one of them:

  4. Hello Dr. Lederer. I am so happy I found this page.
    My Father was diagnosed with IPF few years back. His cough of course is debilitating and severe SOB. 3 weeks ago he was admitted to the hospital with pneumonia and dehydration. He was on IV steroids. He now has cushing syndrome. His legs are so weak and he has edema in one leg. Will these symptoms subside? He is off all steroid treatments at this time but that being said his cough is at it's worse and so is the SOB. He is taking OFEV for around a year. I feel like he is fighting a losing battle and I am going to lose him soon. Any suggestions will help.

    Thank you,