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Scleroderma Lung Disease

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#1 LMS


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Posted 08 July 2008 - 01:53 PM

Hi Everyone,

I have pulmonary hypertension and my recent CT lung scan shows no fibrosis. My DLCO is decreasing based on a recent PFT. My pulmonologist is very experienced in treating lung disease in Scleroderma patients. I can't help but think I should probably be on something for lung fibrosis eventhough nothing is showing on the scan. In other words does a doctor treat the fibrosis before or do they wait until fibrosis is visible on a scan? And what is the probability I will develop lung fibrosis given that I already have pulmonary hypertension? Thanks.


#2 janey


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Posted 08 July 2008 - 03:15 PM

On our pulmonary hypertension page I found a recent study (2007) that states "This is the first demonstration that the reduction in DLCO in PAH-CTD is at least in part related to loss of functional capillary surface area, and is proportional to its severity." I'm no doctor, but it could be that your DLCO reduction could be related to the PH. Here's access to the study abstract if you'd like to review it. DLCO in PH.

Pulmonary fibrosis is normally treated with immunosuppressants like cyclophosphamide or more recently, cellcept. Our you on an immunosuppressant?

Of course if you sypmtoms to not improve or stabilize, please let your doctor know. Hope this helps.

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)

#3 CraigR


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Posted 09 July 2008 - 06:39 AM

You might never develop fibrosis. Like you, I have pulmonary hypertension, but little, if any fibrosis.

The immunosuppressant drugs used to treat fibrosis can have dangerous side effects. Suppressing your immune system can increase the likelihood of infection or cancer - so probably not a good idea to take prophylactically for a condition that you may never get.

A while back there was some discussion about the PH drug Tracleer (Bosentan) possibly also having anti-fibrotic properties. I haven't heard any other news about this. So, if you take Tracleer, you might also get this benefit. Your posting doesn't mention if your taking anything for your PH.


#4 Gidget


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Posted 09 July 2008 - 03:48 PM

You indicate that your DLCO is decreasing. It is my understanding that PFT results can have as much as a 10 point swing do to equipment or technician. As such, the pulmonologist may not react when a PFT shows lower test scores than a previous PFT as it may be do to the difference in equipment or technician which can cause the 10 point swing. You should discuss how your Pul. Doctor evaluates the PFT for a better understanding. I would guess that if your 2nd or 3rd PFT continues to show lower results in excess of the 10 point swing from the original or baseline PFT, that your doctor will then believe that your condition is worsening. It is very important when having PFT tests done, that they be done at the same location as different locations have different equipment which produces different test results. Good Luck. gidget