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Pulmonary Fibrosis

DIP in a 57-Year-Old Who Never Smoked

06/25/2010

Question:

I have just been diagnosed with DIP and have never smoked and am not environmentally exposed to smoke. I had a VATS lung biopsy and the DIP was the diagnosis on the pathology report. I also have recently been diagnosed with psoriatic arthritis (no rheumatoid factor) in the same 2 month period of time. The lung pathology report indicates no relationship between the two diagnosis but my pulmonologist is not readily accepting of the DIP diagnosis. Where do I go from here as now I am not receiving meds and want to get better. Thank you.

Answer:

DIP (desquamative interstitial pneumonitis) is a form of interstitial lung disease when the alveoli (air sacks of the lung) fill up with a type of lung cell called the alveolar macrophages. This condition is very rare in non-smokers and occurs almost always in smokers. 

The diagnosis of all forms of interstitial lung disease relies on a combination of the clinical, radiographic, and pathologic findings. At many referral centers, there will be venues for the clinicians, radiologists, and pathologists to get together to review all of the relevant tests to discuss cases and arrive at a consensus diagnosis. It is important to know where the biopsy was taken and whether the location of the biopsy(s) was in the area of the lung where the most active disease was taking place. 

There are other interstitial lung diseases that can on occasion mimic DIP, and sometimes a second opinion on the biopsy can be helpful. Interstitial lung diseases are very difficult for many pathologists, and so frequently second opinions from one of the relatively few pathologists in the country that specialize in interstitial lung disease can be useful. 

Patients who do have DIP and are smokers will frequently get better with smoking cessation alone. Medications such as prednisone and azathioprine can also be useful in patients with DIP.

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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