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Pulmonary Fibrosis

Pulmonary Fibrosis and Failed DLCO Test

09/14/2009

Question:

I am 25 years old and am a female non smoker. For 2 months I have had sob and purplish nails. I just had a pft and my dlco was significantly decreased. I was at 12 and was supposed to be at 43. I guess that is the 69%. Is the only possibilty an interstitial lung disease? Do those diseases suddenly show up? My pulse ox has been normal. I am so scared that I only have 3-5 yrs to live and my pulmonologist doesn`t have me schedule until mid-October. I can`t wait that long! Could it be something simple that caused the dlco to be that low? I am so young, and have never been around strange chemicals. Please advise.... I am very scared.

Answer:

Purplish nails is the condition called "cyanosis". This usually means that there is a low blood oxygen level, but it can also be seen in a rare blood disorder called "methemoglobinemia" and a more common disorder of circulation called "Raynaud's phenomenon". When associated with a low diffusing capacity (DLCO), then this could be a sign of many underlying conditions including congenital heart disease, pulmonary hypertension, or interstitial lung disease. Interstitial lung disease can occur in patients who are as young as 25, but it is rare this young. Pulmonary hypertension would be a more likely condition at this age.

The first step should be to measure the oxygen when the fingers are purplish and cyanotic. If the oxygen level is normal despite cyanosis, then Raynaud's phenomenon is more likely. If the oxygen level is low, then congenital heart disease, pulmonary hypertension, or interstitial lung disease are more likely; if this is the case, then evaluation and treatment is more urgent than would be the case with Raynaud's phenomenon.

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Response by:

James N Allen, Jr, MD James N Allen, Jr, MD
Clinical Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University