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Current Concepts in Progressive Pulmonary Fibrosis (PPF)

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That's ILA interstitial lung abnormality.

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The progressive pulmonary fibrosis idea is

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very similar to ILA.

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So progressive pulmonary fibrosis is, are gonna be cases

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of pulmonary fibrosis

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that are not idiopathic pulmonary fibrosis.

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So I, as I alluded to

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before, most clinics are gonna have a big

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proportion of their, their interstitial lung disease cases

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be IPF and so almost all cases of ipf

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or maybe it's better, just better say by definition,

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these IPF cases are all gonna get worse.

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And so we know these IPF cases are getting worse,

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but other types of pulmonary fibrosis, some

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of them actually don't get worse.

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The ones that do get worse, these are gonna be patients

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who we, we invoke this idea

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of progressive pulmonary fibrosis.

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And so how do you figure this out

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and how do you make sure sort of define

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that patient is getting worse?

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Well, it's gonna be a combination of clinical symptoms,

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functional abnormalities and radiologic

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progression within the past year.

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And so these patients may benefit from antifibrotic therapy

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as do patients with idiopathic pulmonary fibrosis.

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Again, this is not a diagnosis.

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PPF is not a diagnosis, it's an idea.

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It's sort of taking these non IPF subset of patients here,

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right, saying Oh, these are non IP patients.

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I'm saying, alright, well these subset that are progressing,

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these patients may benefit from this additional antifibrotic

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therapy because we have data to back that up.

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That is the purpose of progressive pulmonary fibrosis.

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So I'm sure you guys see some of the corollaries

1:30

between progressive pulmonary fibrosis

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and the, the idea of interstitial lung abnormalities.

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So again, any type

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of pulmonary fibrosis can be a progressive pulmonary

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fibrosis except I-P-F-I-P-F

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by definition is gonna be getting worse.

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