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Scleroderma and lung disease vs Just lung disease

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#1 Ron


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Posted 28 May 2015 - 02:49 PM

I was wondering if anyone has found any evidence of slower progression of lung disease with Scleroderma than just someone with a lung disease like Pulmonary Fibrosis.

My scleroderma history is long 28 years, Raynauds started in 1984, scleroderma diagnosed in 1987, with slow progression, my dry cough started in about 2008, and I attribute that to my pulmonary fibrosis diagnosis 3 years ago.

I am wondering if the survival rate of 2-5 years for pulmonary fibrosis applies to scleroderma and people without scleroderma.

Does that make sense?

#2 Shelley Ensz

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Posted 28 May 2015 - 05:40 PM

Hi Ronald,

That's an excellent question and I'm glad you asked it. I think you might even like the answer (well, considering, and compared to.)

Generally speaking, idiopathic pulmonary fibrosis (IPF, for those who don't know, means pulmonary fibrosis without a known underlying cause) has a much worse prognosis than the pulmonary fibrosis of scleroderma. See this article for a more thorough explanation:

Lung Involvement by the University of Michigan Scleroderma Program.

I've known many people with scleroderma who have lived with pulmonary fibrosis for many years. It frequently stabilizes, even without any treatment, and does not have the average horrible life expectancy as that of IPF.

Warm Hugs,

Shelley Ensz
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#3 Joelf


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Posted 28 May 2015 - 07:19 PM

Hi Ronald,

When I first developed the symptoms of lung involvement, I didn't attribute this to Scleroderma, but very foolishly googled my symptoms and then convinced myself that I had Idiopathic Pulmonary Fibrosis, thereby scaring myself witless. My consultant actually sat me down, looked me in the eye and told me in words of one syllable that I "did not have IPF!!" (I now realise the folly of looking up symptoms on the internet.....a little knowledge is a dangerous thing!! :wink: )

As Shelley has said, the prognosis is not very good for IPF, but Scleroderma induced non specific interstitial pneumonia (NSIP), which I have, is probably the "best" sort of lung disease to have, with the most favourable outlook.

Kind regards,

Jo Frowde
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#4 Ron


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Posted 29 May 2015 - 05:43 AM

Hi Shelley and Jo,

That's very encouraging. My last PFT was in January 2013. The results were "moderate restrictive defect." I called the clinic and they said they don't give out percentages for lung damage. They use, mild, moderate and severe.

I go for my next PFT on June 25, 2015. I know it will be worse than the one in 2013 as I now have shortness of breath. But that article puts my mind at ease somewhat.

Thank you!!