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Interstitial Lung Disease, Pulmonary Fibrosis

4 posts in this topic

I was wondering what the difference is between these conditions. I have been diagnosed with ILD and PH. Are ILD and PF different things or are they the same? What about PH and PAH? I see my pulmonologist next week but I was hoping I could have some answers before I go. Thanks.



ISN Artist

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I'm sorry to hear that you've been diagnosed with both ILD and PH. I, too was diagnosed with both a few years ago. We caught it early so both have been stopped in their tracks and have not worsened. In fact, the PH has improved through a drop in the pulmonary artery pressure. So to answer your question - Yes they are different.


ILD or Interstitial lung disease is also called pulmonary fibrosis. Here is a quote from the ISN page on pulmonary fibrosis.

Pulmonary (lung) fibrosis, is a scarring of the lungs, and is the consequence of untreated pulmonary inflammation (alveolitis). It is often also referred to as interstitial lung disease. For me diagnosis was made with a CT scan and pulmonary function tests results.


"Pulmonary Hypertension is a high pressure in the arteries between the heart and lungs or pulmonary arteries. It is very difficult to detect elevation in pressure in these arteries. Pulmonary Hypertension is a type of vascular disease that can be found in scleroderma patients." ISN The best way to diagnosis PH is with right heart catheterization.


Because they are different, the treatments are also different. I'm on Bosentan for the PH (which has also helped my Raynaud's - WhooHoo) and the fibrosis is treated with immunosuppressants, such as cytoxan or cellcept. The ISN pages on ILD and PH have lots of information. Please let us know what you doctor says. We're here for you.

Janey Willis

ISN Support Specialist

(Retired) ISN Assistant Webmaster

(Retired) ISN News Director

(Retired) ISN Technical Writer for Training Manuals

International Scleroderma Network (ISN)

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Hi Miocean,


I'm sorry you have joined the PH/PF club after getting your kidney problems sorted out so nicely (happy 2-month anniversary of your transplant!). I got my diagnosis of systemic sclerosis/pulmonary fibrosis/pulmonary hypertension all on the same day, 3-1/2 years ago. The initial diagnosis was made via CT scan and both bubble and regular echocardiogram, followed up a bit later with a right heart catheterization. I'm on CellCept (working well) and Letairis (ditto!). I haven't seen any improvement on the Letairis and it hasn't improved my Raynaud's, but hey, stable is very good.


Janey has given you some good links. I think the only thing I have to add is that if you get a chance to see a pulmonary hypertension specialist, it would be a good thing. I would guess that your transplant team would work closely with your other specialists to make sure nothing endangers your transplant and would do a thorough medication review.


It's a scary diagnosis, to be sure. We'll all be keeping you in our thoughts.

Jeannie McClelland

(Retired) ISN Director of Support Services

(Retired) ISN Sclero Forums Manager

(Retired) ISN Blog Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Artist

International Scleroderma Network

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Hi Miocean


As the others have said ILD and PF are names for the same condition and I am also a member of that particular club!! :( However, thankfully I don't have PH and interestingly I was reading the other day on The Scleroderma Society site about the various different autoantibodies. I have been tested positve with Anti-Pm/Scl which apparently are associated with Sclero/Myositis overlap and patients with these antibodies carry a high risk of developing PF but not PH which describes my condition exactly!! ;)


I am also on Azathioprine (immune suppressant) and Prednisolone to reduce the imflammation on my lungs and I'm happy to say it seems to be very successful. :)


So sorry to read that you've been diagnosed with both PF and PH especially as your kidney transplant has been so successful.

Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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