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Practical Approach to Pulmonary Fibrosis

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Let's talk about that practical

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approach that I mentioned before.

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So if you have someone pulmonary fibrosis, so you know,

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what is pulmonary fibrosis, reticulation,

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these tiny little lines in lungs, traction,

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bronchis honeycombing.

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I think everyone knows that they're dealing with a case

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of pulmonary fibrosis on HR ct,

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even if they don't have any formal training.

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Why is that? I don't know. It's just, it seems obvious even

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to medical students when I show them an HRCT

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that has pulmonary fibrosis on them,

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even if they don't have never seen pulmonary

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fibrosis before.

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And I haven't really taught them about pulmonary fibrosis.

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If I show 'em an HRCT, oftentimes they'll see things like,

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oh, that looks like scarring, or something like that.

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Which is essentially what fibrosis is.

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It's a, it's a progressive type of scarring of the lungs.

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And so I don't know what it is,

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but there's something about it

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that I think is quite characteristic.

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But once you know it's pulmonary fibrosis,

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that's actually the hard part.

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Now what do you do with it?

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So I think this algorithm will help you quite a bit.

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And so the first step is to look at that HRCT

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and try to determine is this a typical UIP

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or a probable UIP pattern on ct?

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If it is, as a radiologist, you're done.

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You could drop that mic, you're done.

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You've achieved the answer from a radiological standpoint.

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Why is that? I'll tell you in just a little

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bit why that's the case.

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But typical UIP, probably UIP,

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when we see this pattern, we should be quite happy.

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'cause this is actually when we are most accurate in terms

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of predicting what the UIP pattern would be had we be able

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to do X explan and look at the whole lung.

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If it's not typical UIP

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or probably UIP, you gotta go to the next step.

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You gotta ask yourself, is this a high confidence pattern?

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Like I will eat my hat if it's not this diagnosis kind

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of pattern of nonspecific interstitial pneumonitis,

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hyper pneumonitis or sarcoidosis.

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You're gonna hear me interchange pneumonia and pneumonitis.

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A lot of people do this and so they are interchangeable.

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But I will say, I think the favored P

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nomenclature is to call it pneumonia.

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So nonspecific interstitial pneumonia, same thing

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as nonspecific interstitial pneumonitis.

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Okay? But you're gonna hear me kind

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of use them interchangeably because I just

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can't, I can't keep it straight.

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Uh, just, it just comes out naturally.

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If you can, you're, again,

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we're done from an imaging standpoint.

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You drop the mic and you say,

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this is a high confidence pattern for one of these different

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alternative disease categories, NSIP, HP or sarcoidosis.

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And then step three is, well,

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you gotta kind of have to punt.

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So if it's not one

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or two, then it's probably something weird.

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And so within the framework

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of pulmonary fibrosis diagnosis on imaging,

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you have two categories that you can leverage

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to address these cases.

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So one is the alternative diagnostic category.

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These are cases where you're essentially saying,

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this just does not look like UIP does not feel like typical

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UIP or probable UIP.

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This feels like something else.

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Um, and maybe you just can't put your finger

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on exactly what it is.

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And then there's the indeterminate for UIP category,

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which is a great category.

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The indeterminate for UIP category essentially is saying,

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I have no idea what this is.

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I don't think it's UIP,

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but I'm not confident that it's something else.

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I don't know what pattern this is.

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This is kind of thrown up your hands.

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And so this pattern is often invoked in cases

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of very mild pulmonary fibrosis where you're like, Hmm,

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I don't really, is this early NSIP?

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It's early UIP. It could be early hp.

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I mean, there's a little bit of air trapping.

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You're just not quite sure.

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So that's good for interterm of UIP

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or for more advanced cases

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where you just really have no clue.

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And so I call it punting.

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Again, you're not really sure what

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to do, but this is helpful.

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If a radiologist says, I have no clue what it is,

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then this actually acts more like this alternative

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diagnostic category,

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like a non UIP category than it does UIP.

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I wanted to just underline this fact.

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I know I mentioned this before. Remember,

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multidisciplinary diagnosis is the gold standard in

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achieving diagnosis in interstitial lung disease

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and pulmonary fibrosis.

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It's just the way it is. And why is this?

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Is 'cause we're not talking about one single little nodule

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that you're sticking a needle and you're doing biopsy on.

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Right? In those cases, you can be pretty sure

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whatever the pathologist tells you is gonna be accurate.

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Like, like there's no way that if you,

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you cord out a big hunk of a pulmonary nodule, he came back

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with lung cancer, there's no way the radiologist's gonna

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come back to the pathologist and the pulmonologist

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or oncologist and say, oh no, this is not lung cancer.

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This is something else. No way, right?

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Pathology, that's gonna be the gold

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standard diagnosis in that setting.

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But an ILD, because you have a diffuse lung disease,

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you can either sample if you're doing surgical lung biopsy

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areas that are too fibrotic,

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or maybe just the fact that it's,

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it's such a diffuse process, you're gonna sample areas

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that maybe don't give you the right answer.

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And so there is some data out there,

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a little older data now,

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but it shows that if you get a pathologist

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and you give them their pathological slides

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and then you bring the pathologist back to an MDD setting,

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multidisciplinary diagnostic setting,

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and then everyone discusses the cases,

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the pathologist will change his

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or her mind in about 20% of cases, 20% of cases.

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That's crazy, right? So no way would that happen

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with a pulmonary nodule.

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Again, we're gonna trust what the pathologist says

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by the setting of interstitial lung disease

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and pulmonary fibrosis.

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MDD is really what we're gonna be trusting.

Report

Faculty

Jonathan H. Chung, MD

Professor of Radiology and Division Chief of Cardiothoracic Imaging

UCSD - University of California San Diego

Tags

Syndromes

Non-infectious Inflammatory

Lungs

Idiopathic

Drug related

Chest CT

Chest

CT