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Hi Jennifer,

 

Good question. I think the answer is No. It's my understanding that only a small percentage of people with pulmonary fibrosis go on to develop pulmonary hypertension.

 

If I remember correctly, a majority of people with systemic scleroderma eventually develop pulmonary fibrosis (to a greater or lesser degree), but only a small percentage develop pulmonary hypertension.

 

For the actual facts of the matter, visit: Pulmonary Fibrosis and Pulmonary Hypertension. And, of course, please correct me if I'm wrong, as I often am! I have no medical training at all, bladda bladda bladda....


Warm Hugs,

 

Shelley Ensz

Founder and President

International Scleroderma Network (ISN)

Hotline and Donations: 1-800-564-7099

 

The most important thing in the world to know about scleroderma is sclero.org.

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Thank you Shelley!! Always a life saver for me! I think I am losing my memory...I mean, I am getting older and now I have a husband to contend with...haha. I know I've asked the same question a hundred times but why can't I ever remember things?? It must be a combination of aging and medications.

Anyway thanks and have a great rest of the weekend!

Jennifer

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Hi Jennifer,

 

Excellent question! As you know I have ILD and tomorrow I find out the results of my echo. So I may be back with a diagnosis of pulmonary hypertension too. I'll let you know....


Lisa Bulman

(Retired) ISN/SCTC List Coordinator

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Fundraiser

International Scleroderma Network (ISN)

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Lisa,

I will be thinking of you tomorrow. Please post asap and let us know how the diagnosis went. My thoughts are with you. ILD is just no fun. I'll have more pfts at the end of March. So anxious to see the changes.

Take Care,

Jennifer

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Will be thinking of you Lisa. Hope your echo goes well. I am still working out the results of mine with Borderline pulmonary hypertension. Hope you get great results. Susie54

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Hi Jennifer!

 

I think I read something similar to what Shelley said. In fact, I think that people with diffuse have a greater chance of developing ILD but that people with limited have a greater chance of developing PAH. Hence all those programmed screening tests we must undergo (echo's pft's, etc.)

 

The good news is that there's lots of new medications out there that weren't available 6-7 years ago to treat these issues. We're in a lot better shape now that only a few years ago.

 

Thank goodness for modern medicine!

 

Lisa, please let us know how your tests went.

 

Best of luck,

 

Claudia

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Jennifer,

I have both mild PH and ILD. I'm taking Bosentan for the PH and since starting it my PA pressure has dropped from 42 to 32 on the ECHO. I get another ECHO on March 1. A HRCT showed fibrosis in the lower lobes back in November so I was put on oral Cytoxin for the ILD and as my immunosuppressant. I went off it when I went septic last month and also dropped the dosage on the Bosentan in half. I did get back to full dosage on that last week. On Wednesday, my rheumatologist and I discuss going back on Cytoxin (NOT) or another drug, possibly Cellcept.

 

As far as a relationship between the two, I couldn't find anything either. In one of the links that Shelley provided I did find this article:

 

Scleroderma Patients with Combined Pulmonary Hypertension and Interstitial Lung Disease

 

It didn't mention any correlation between the two but instead saw them as different pathological processes. The study's objective stated

"Most studies differentiate scleroderma associated pulmonary hypertension and interstitial lung disease (ILD) as 2 separate pathological processes, concentrating on one or the other; however, many patients have both conditions. We studied the demographics, clinical features, and prognosis of individuals with both vascular and interstitial lung disease."

 

Lisa, I'll have my fingers crossed for your ECHO showing a low PA pressure!

 

Big Hugs


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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Janey,

 

I was wondering at what point they treated your pulmonary hypertension. What were the pressures that they put you on the Bosentan? Mine was 28-33 showing borderline. Sounds like you have been through alot. I hope things improve for you.

 

Susie54

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Susie

My ECHO is August had a PA pressure of 42 and that's when I was started on Bosentan. My previous ECHO in January was a PA pressure of 35 but my pulmonologist at the time, said "no worry" even though it had continued to increase over the past year and a half from 18! Thank goodness I found a new pulmonary doctor!

 

Big Hugs,

Janey


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 everyone,

I'm back from the dr.'s. First we went to Grace's dr and had her stitches removed. She did great and it was a breeze.

 

We then hurried into Boston, in the snow, to get to my pft. My numbers are good, 1% better than last time but really no change. I'll take it, as long as they are not worse. He said I have good capacity and it is not progressing at this time. Looks like the chemo is holding it at bay.

 

We then discussed my echo from two weeks ago. He said it is pretty much unchanged since 12/05. BUT my PA pressure is 35. He said borderline and we are not going to worry unless I am symptomatic. I did tell him that I have been lightheaded and dizzy lately. I am going to do a Holter monitor for 24hrs to see if my arrythmia is acting up again. If that shows nothing then I am not sure where to go from this point.

 

Hugs,

Lisa


Lisa Bulman

(Retired) ISN/SCTC List Coordinator

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Fundraiser

International Scleroderma Network (ISN)

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Hi Lisa,

 

I am glad that you are back safe and sound and didn't get stuck in the snow! It sounds like your PFT numbers are holding steady...yeah!! That is great! I just hope you can figure out why you have been feeling lightheaded and dizzy and it is someting minor and treatable. When do you start the 24 hr holter monitor?

 

Warm wishes,

Heidi

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Lisa,

Congratulations on the PFT results! It's always good to see NO CHANGE, but to also get a little improvement - we'll take it! It's going to be interesting to see the results from the Holter monitor. I hope your arrhythmia isn't acting up again. I go in tomorrow to the cardiologist because my pacemaker check last week showed that I've had over 800 atrial fibrillations in the past 6 months and within the past two months they have become more frequent and longer. Well, imagine that! Knowing this doctor, rather than seeking the "cause", he'll probably just want to go up on my meds.

 

I can't believe Grace is getting her stitches out already! She MUST be doing GREAT! Glad you both got home safely.

 

Big Hugs,

Janey


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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Lisa,

If you dont' mind sharing, what were your numbers? I am thrilled for you that they are basically the same and not worse!! Congrats!!! Hey, I'm also wondering...will you eventually have to have another round of chemo, or how does that work?

I hope you have a great evening.

Jennifer

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HI guys,

Thanks for thinking of me! I am waiting for them to call me about thr holter monitor. I'll probably do it next week because after my trip to NY with grace, then into Boston today and we are supposed to go skiing this weekend (actually I go shopping at the outlets) I am sick of driving.

 

Jennifer I don't mind you asking at all! I have only the numbers my doctor really looks at:

FEV1 2.67 liters

DLCO 13.3 or 63%

 

As for the chemo, we really don't know if I will need it again. It is all so new to everyone, my doctor has no idea how long it will last. So far so good since I finished my treatments last June.

 

I forgot to mention my liver function numbers were almost back to normal! :D

Last time we I had them done they were 544 and 299!


Lisa Bulman

(Retired) ISN/SCTC List Coordinator

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Fundraiser

International Scleroderma Network (ISN)

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