Restrictive Lung Disease or what?

[myproblem]

30 yo F. Since age 15, shortness of breath upon exertion. At 18 was told I had asthma because there is a family history, but no testing was done. Dr.'s have always said that my lungs sound clear. At 19 I tried to get into an Asthma Study, spirometry was 119% of the predicted volume, and I failed the methacholine challenge twice. (In otherwords, it did not induce an asthma attack.) I was unable to participate in the study because I did not have normal asthma reactions. At this point I began to doubt the diagnosis, but I did not have insurance most of the time. Over a period of 11 years I tried so many different steroid inhalers (vanceril, serevent, asthmacort, flovent, advair, to name a few), nothing gave much relief. Albuterol has stopped working. Breathlessness has gotten worse, I have a hard time even if I walk fast, or lift something heavy. Often have breathlessness at rest. A year ago Dr. ordered pulmonary function tests. (Could not get methacholine challenge at this hospital.) Spirometry was 116%, albuterol did nothing whatsoever. Lung volume was only 60% of predicted. Hospital report suggested a neuromuscualr problem, but my Dr. said it is more likely restrictive lung disease because I have MCTD (Lupus, and Scleroderma). He said that the high spirometry values suggest that my lungs are sort of stretched out. The pulmo. treated me like a hypochondriac, and told me shortness of breath was just a sensation I was having. When I pressed him, he told me to see a neurologist. Is this RLD? What can I do to help it? Is there any treatment? How can I be sure of the diagnosis?

[National Jewish]

The evidence suggests that asthma is not the problem.  However exercise-induced asthma could still be an isolated phenomenon.  If your lung volume truly is 60% of predicted, then you almost certainly have restrictive lung disease.  That could be the cause of your shortness of breath.  As your doctor has stated it could be restrictive lung disease due to your mixed connective tissue disease (MCTD).  A rheumatologist would be the most helpful specialist to determine if your MCTD is affecting your lungs.  Please read our information about lupus at http://www.nationaljewish.org/disease-info/diseases/rheum/lupus/index.aspx and scleroderma http://www.nationaljewish.org/disease-info/diseases/rheum/scleroderma/index.aspx to learn more.

Interstitial lung disease (ILD) is a type of restrictive lung disease.  Usually a high resolution CT scan of your lungs will identify if ILD is affecting your lungs.  You may also need an echocardiogram.  Please read our information about ILD at http://www.nationaljewish.org/disease-info/diseases/rheum/ild/index.aspx to learn more.

There are many other causes of shortness of breath including heart disease, pulmonary hypertension and recurrent pulmonary emboli, to name a few.  If your doctor(s) do not take your symptoms seriously, then you need to find a doctor who will.  A pulmonologist would be the most helpful specialist to sort out if a lung problem is causing your shortness of breath.

[myproblem]

One more thing, my last lung x-ray showed some scar tissue in the lower portion of one lung. I don't know about previous x-rays.

[Between]

Hi,
You said your diffusion is 60%, and the hospital report said muscular problem. Did they state what your dlva was? I know lupus can cause restictive problems from what I read, but a low dlco can be many things. Do you have a copy of your report and what the dlva was?

[Sunny602]

I feel your frustrations...I went through about the samme thing you did regarding the asthma diagnosis, (Iwas about your age BTW) I was treated heavily with steroids and other meds. Yes I wheezed, but after awhile, I could barely walk and breathe and knew something wasn't quite right, it didn't feel like asthma. So, when the docs sent me to a teaching university where they did very comprehensive tests including the full set of PFT's my pulmo was surprised to see that my volumes were low, FEV's too as well as FVC along with other indicators that made him scratch his head and say "neuromuscular". My ABG's or blood gases weren't great either so I was put on O2, eventually BiPAP and now a a ventilator. My diaphragm does not function...that was a few years back. This was called restrictive lung disease, severe due to neuromuscular disorder. Your spirometry results are pretty good, but those volumes are low.I sure hope they figure out what is wrong...
FAst forward to now, the docs have discovered that I have CF (cystic fibrosis) on top of the neuro problems, so I have mixed results....It is a very long story how they came to that diagnosis, it was mainly the persistance of my primary doctor that led to this diagnosis as well.

Please keep us updated...

My thoughts are with you, Sunny

[myproblem]

I don't have a copy of the PFT's, but I guess I should get it. I just know that it is not my diaphragm, because my spirometry is good. It is just that my ability to draw in air is reduced, indicating a reduced lung volume. That is how my internist described it.

[G.P.]

My husband only had one number on his spirometry that was mildly reduced, yet was found to have a paralyzed diaphragm.  He had severe shortness of breath, but showed an almost normal blood oxygen level.

His right lung volume showed on an X-ray to be mildly reduced, and the right diaphragm elevated.  Many doctors looked at the X-ray before someone finally caught it.