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Case: UIP with NSIP Elements in IPAF

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0:00

So here's another somewhat complex case,

0:03

but I think that we, this can be broken down pretty easily.

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So in scrolling through, clearly there are areas

0:09

of reticular abnormality and mild ground blast opacity,

0:13

but more concentrated in the lung periphery than

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central aspect to lungs.

0:17

And there's definitely a basar gradient.

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Look at this beautiful traction bronchis like, oh,

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it's beautiful, isn't that?

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And so what do I say about NSIP, right?

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Remember the NSIP can give you this very exuberant traction

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bronchiectasis almost looks like a beaded appearance here.

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So NSIP likes to do that.

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And the ground lasts opacity plus the reticulation.

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That also we can see an NSIP,

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but we see a lot of these cystic abnormalities.

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And so these cysts, at least when we get down

0:46

to the Coran angles, they meet the definition

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for honeycomb cyst.

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So someone might be tempted to just kind

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of call this UIP.

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And I think if someone called this UIP, they would be

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70%, right?

1:01

'cause it is, if you were to biopsy this, almost

1:03

as surely you get UIP back.

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But it doesn't capture the true pattern

1:07

of diffuse lung disease here, does it?

1:09

Because there are elements of both NSIP and UUIP here

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and that actually is really a known phenomenon.

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So NSIP, as it becomes more

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and more severe, can start to look more

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and more like UIP instead.

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In fact, end stage fibrotic NSIP will look very,

1:27

very similar to UIP.

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And that's probably what's going on in this case.

1:31

This is, if we were to find, if we're able to get a hold

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of like the earliest CT scan possible,

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let's say from like 10 years ago at an outside hospital,

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I bet you this looked like classic NSIP.

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Okay? And so that's, that's quite helpful here.

1:44

So if you have someone

1:45

with this combined N-S-I-P-U-I-P pattern,

1:48

well now we're talking, right?

1:50

This is great because we know that NSIP,

1:54

that's gonna be more common to in the setting

1:57

of secondary lung disease.

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So things like connective tissue disease, fibrotic, hp

2:01

or drug related pulmonary fibrosis.

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And so even though there are UIP elements here, the fact

2:08

that we feel like this emanated from NSIP makes us think,

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okay, this is likely not idiopathic pulmonary fibrosis.

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We should dig deeper. We should talk to our pulmonologist.

2:18

We talked to our rheumatologist

2:19

and try to really figure out

2:20

what is the best diagnosis for this patient.

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And probably the most helpful thing would be let's get hold

2:25

of that CT from five or six years ago or 10 years ago.

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'cause this pulmonary fibrosis is not mild.

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This patient was dyspneic a long time

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before they came to your hospital and

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or your imaging center.

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And so you have that opportunity to make that diagnosis.

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Small pearl here, if you have someone

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with significant pulmonary fibrosis like, like this,

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very commonly from a diagnostic standpoint,

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this is not gonna be the most helpful CT scan for you.

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From a diagnostic standpoint.

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Obviously from prognosis it's helpful

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'cause you could tell how much lung is involved. But in

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Terms of of diagnosis, the most helpful CT very often is

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the earliest CT scan on your pacs

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or the ones that you get a hold of.

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And why is that? Is

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because as I, I alluded in this case almost all types

3:12

of pulmonary fibrosis, as they get more

3:13

and more severe, they start to look more and more like UIP.

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And so there's gonna be more

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and more overlap in terms of that disease pattern

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that you're seeing and the UIP pattern.

3:24

So try to find that earliest CT possible,

3:26

it'll pay dividends.

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

Chest CT

Chest

CT