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Interstitial Lung Disorders

Chest radiographs are helpful in defining the person may actually have a interstitial lung disorder. They may be read as normal in as much as 10% of cases. Thatís because the amount of interstitial markings varies with the kilovolts used when they take the x-ray and if

Pleural plaques, particularly calcified pleural plaques, are very suggestive of asbestos exposure. Even someone with a vague asbestos exposure history. Bilateral hilar adenopathy is probably not surprising that that is fairly suggestive of sarcoidosis and there are some diffuse honeycombing patterns in histiocytosis X which can be kind of characteristic but we

This was a young woman, 33.  She presented with cough, malaise, shortness of breath and her x-ray shows adenopathy, adenopathy, adenopathy. So the immediate diagnostic impression was probable sarcoidosis given that she was a young woman with this bilateral hilar adenopathy and a rather acute onset illness. Yeah. She was 33. Rapid onset of her disease. Her lung function studies, when we first saw her, were in the normal range. You see most of her values here are on average around 90% and predicted which would be within the normal range. A few months later, after a brief course of treatment, the numbers actually improved. Her vital capacity improved about 400 or 500 cc. Her DLCO improved about 10%. So she had about a 10% improvement in her lung function, actually, even though it was fairly normal to begin with.

This one, we went to a transbronchial lung biopsy on her. Transbronchial lung biopsies are particularly good at diagnosing sarcoidosis because we just need to find a few granulomas like we see here. Here are some normal alveolar walls. Not a lot of the inflammation in this biopsy but you see these well defined rounded granulomas with a few lymphocytes around the rim Ė epithelioid cells. I donít remember if weíve got any giant cells in here. Maybe one there and maybe one there. Some giant cells at the center. So this case was fairly straightforward. We would send cultures of our bronchoscopy specimens for acid fast bacilli or mycobacteria just to make sure but given that those cultures come up negative and the special stains donít show any acid fast bacilli, then the diagnosis basically comes down to sarcoidosis. We did give her a brief course of steroids so she could go back to work and her chest x-ray looked much improved. The adenopathy is gone. Then I think about a year later she came back for some follow up. She hadnít had any further treatment and the adenopathies were really completely gone then. She probably would have spontaneously resolved on her own without any steroids but she wanted to go back to work and was willing to tolerate steroids for four weeks to accelerate the resolution of the process.

Then we had another young woman who did present with rather progressive shortness of breath. She worked in a factory here in town. It was a plastics factory and she was having a lot of trouble working. Again, these donít project well but thereís a lot of disease here in her lower lobes. You can see all this honeycombing and a lot of stuff here on the lateral view on her chest x-ray. Hereís one of her CT scan cuts and you can see her lung parenchyma is not normal. This is fairly normal here but you can see posterior in the bases a lot of fibrotic changes with a lot of little honeycombing in here spread out where there is destroyed lung, fibrotic stranding and there were other cuts further up in the lung which looked quite similar.

Well, I tried to make a diagnosis of scleroderma on her because I thought the skin on her hands was quite thick. Maybe that was from her manual labor but the rheumatologist wouldnít make a diagnosis of a rheumatologic disorder on her. This is her lung biopsy and Iím not showing you the honeycombing area which would just show complete fibrotic change. You can see some alveolar walls here and then you see these big areas of fibrosis stuck in the alveolar walls. None of this is normal, of course. Obviously, her gas exchange Ė thereís myofibroblasts in here Ė itís pretty messed up. I didnít have any further followup. She was 37 or 38 and she died on the lung transplant list. So a diagnosis of probable idiopathic pulmonary fibrosis has a lot of meaning for a person like that. Itís often a fatal diagnosis. This is a case where we felt it necessary to go to an open lung biopsy because at her age, it was seriously important to her.

If we got a patient like this who was elderly, or letís say, someone past the age of retirement, and you got this kind of CT scan, and you might even have a positive ANA and a negative rheumatoid factor, a lot of clinicians probably wouldnít biopsy such a patient in that the treatment is not particularly good. High dose steroids, sometimes you use Imuran or Cytoxan and so some of these patients weíre willing to make a clinical diagnosis of idiopathic pulmonary fibrosis. If theyíve got the crackles, theyíve got the physical findings, theyíve got this high resolution CT scan, you donít necessarily go to an open lung biopsy. But on the patient we were showing here today who is a young woman where there are a few other diagnostic possibilities like lymphangiomyomatosis or histiocytosis-X and this woman was African American, we thought this might be end stage sarcoidosis which would have a better chance or at least sarcoidosis would have a better chance of responding to steroids. So we went to a biopsy in a younger person. In an older patient where some of these disorders like HX and LAM arenít so common, we might not go to an open lung biopsy. But that is fairly dependent upon the different clinicians in how that goes.

So biopsies for interstitial lung disorders. Video assisted thoracoscopy (VATS) is like arthroscopic knee surgery or laparoscopic cholecystectomies. Our thoracic surgeons were using the same scopes that they use in the abdomen but now they have their own which are a little longer designed just for the thorax. But itís quite a bit easier than the traditional open lung biopsy because the surgeon usually

Transbronchial lung biopsies. These small biopsies, which are often only several millimeters big, can be diagnostic for sarcoidosis, cancer or infections. I mean, if you see the bugs, you see the bugs. If you find some cancer cells, then youíve got cancer. If you find a granuloma, you find granuloma. But you can imagine here, a transbronchial lung biopsy, I might stick in and just get, you know, this piece of the lung which isnít going to really give me the full flavor of whatís going on. When you are talking idiopathic pulmonary fibrosis and some of these other disorders, even this field is limited. The pathologist would look at whole bunches of fields of the biopsy in order to feel comfortable with this diagnosis. If your pathologist will diagnose idiopathic pulmonary fibrosis based on these transbronchial lung biopsies, Iíd get another opinion because ours wonít. If they see a couple of granulomas that are fairly straightforward, they will go okay, sarcoidosis but almost everything else, they say "Well, weíre going to hedge." Because if theyíve

Now there are some pulmonologists who believe that since it can give us a diagnosis of some things and itís easier and itís an outpatient procedure that we should do that first. If we donít get a diagnosis, then we go to VATS. In selected patients, I will skip this test and go right to VATS if I really think thatís where the money is going to be. Thatís where weíre going to get a diagnosis.

Bronchoalveolar lavage can be helpful. It can evaluate the alveolitis, the types of inflammatory cells that are there that can sometimes be predictive. Gallium-67 scanning was popular for awhile but I donít see people using it very much anymore. Then, of course, whether