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---
format:
revealjs:
logo: images/circle-cropped.png
link-external-newwindow: true
theme: [dark, mycustom.scss]
width: 1600
height: 900
slide-number: c/t
code-line-numbers: false
chalkboard: false
editor: visual
---
<br> <br>
<h1>Geography of Chronic Interstitial Lung Disease - A 2024 update</h1>
<br>
<h2>Howard Mann, M.D.</h2>
<h2>University of Utah School of Medicine</h2>
<br><br><br><br>
<h2>`r fontawesome::fa("envelope", "white")` howard.mann\@utah.edu</h2>
<h2>`r fontawesome::fa("link", "white")` [howardmann.us](http://howardmann.us)</h2>
<br> <br> <br>
<h2>URL: howardm.github.io/GeographyofInterstitialLungDisease</h2>
{.absolute top="325" right="300" width="300"}
## Content
- Imaging features of common chronic, fibrosing interstitial disorders
- Sarcoidosis and Fibrosing Pneumoconioses
- Usual Interstitial Pneumonia
- Fibrotic Non-Specific Interstitial Pneumonia
- Fibrotic Hypersensitivity Pneumonitis
- Smoking-Related Interstitial Fibrosis
- Unusual causes of chronic interstitial fibrosis
- Familial Pulmonary Fibrosis
- Short Telomere Syndromes (Telemeropathies)
- Surfactant protein (SP)-C deficiency
- Pleuropulmonary Fibroelastosis
- Interstitial fibrosis with non-emphysematous lung cysts
- New concepts in Interstitial Fibrosis
- Interstitial Lung Abnormalities
- Progressive Pulmonary Fibrosis
- LIP -- R.I.P.
## Sarcoidosis
::: columns
::: {.column width="30%"}
Chronic fibrosing sarcoidosis -- Scadding \[criteria\] Stage IV
- Stage 0: no lung involvement
- Stage I: hilar enlargement alone
- Stage II: hilar enlargement plus interstitial lung disease
- Stage III: interstitial lung disease alone
- Stage IV: lung fibrosis
:::
::: {.column width="70%"}
{fig-align="center" width="95%"} <br>
- upper-lung predominant: typically symmetric, suprahilar opacities
- large opacities associated with surrounding architectural parenchymal distortion-cicatrization atelectasis
- often associated with small nodular disease in adjacent lung and nodal calcifications
:::
:::
## Complicated Inorganic Dust (Coal-Silica) Pneumoconiosis
::: columns
::: {.column width="35%"}
- *very* similar to sarcoidosis
- occupational history is obviously very important
{fig-align="center" width="100%"}
:::
::: {.column width="65%"}
{fig-align="center" width="100%"}
<br>
::: {.callout-note icon="false"}
## [Some useful findings that may help to distinguish this from sarcoidosis]{style="font-size: 25px;"}
- cicatricial emphysema (caveat: circumscribed, round cystic spaces may occur in sarcoidosis)
- nodal calcifications typically limited to regional hilar-mediastinal nodes
:::
:::
:::
## Accelerated Fibrosing Silicosis
<br>
::: columns
::: {.column width="20%"}
- often described in workers manufacturing engineered stone products
- relatively rapid development of extensive fibrosis
:::
::: {.column width="80%"}
{fig-align="center" width="100%"}
{fig-align="center" width="90%"}
:::
:::
## Usual Interstitial Pneumonia
<br>
::: columns
::: {.column width="30%"}
- the most common form of progressive interstitial fibrosis
- the manifestation of "end-stage fibrosis" in many conditions:
- idiopathic (IPF)
- connective tissue disease; especially rheumatoid arthritis
- chronic inhaled dust exposure -- asbestosis
- drug toxicity
- familial pulmonary fibrosis and genetic syndromes
- it may occur with other forms of fibrosis, e.g., in fibrotic hypersensitivity pneumonitis
:::
::: {.column width="70%"}
{fig-align="center" width="100%"}
:::
:::
## Honeycombing and traction bronchiolectasis in UIP CT patterns
<br>
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::: {.column width="50%"}
{fig-align="center" width="100%"}
<p style="text-align: center;">honeycombing</p>
:::
::: {.column width="50%"}
{fig-align="center" width="85%"}
<p style="text-align: center;">traction bronchiolectasis</p>
:::
:::
## The evolution of honeycombing in UIP
<br>
A plausible explanation
{fig-align="center" width="85%"}
## Don't agonize over honeycombing versus traction bronchiolectasis!
<br>
::: columns
::: {.column width="30%"}
* UIP is present on biopsy in ~ 85% of patients with the Probable UIP CT pattern
* in general, patients with the Typical and Probable CT UIP patterns are *not* subjected to surgical lung biopsy
:::
::: {.column width="70%"}
{fig-align="center" width="85%"}
:::
:::
## An updated algorithm for the diagnosis of IPF
{fig-align="center" width="90%"}
## If you insist on agonizing about honeycombing!
Some helpful distinctions in honeycombing of UIP
<br>
{fig-align="center" width="85%"}
## What's the pathologic diagnosis ?
<br>
::: columns
::: {.column width="50%"}
{fig-align="center" width="85%"}
:::
::: {.column width="50%"}
{fig-align="center" width="85%"}
:::
:::
## The evolution of Fibrotic Non-Specific Interstitial Pneumonitis
<br>
::: columns
::: {.column width="30%"}
Findings
* extensive traction bronchiectasis within diffuse hyperattenuation abnormality (GGO)
* absent or minor subpleural reticulation; no traction bronchiolectasis; no honeycombing
Most common clinical association: connective tissue disease -- particularly scleroderma
:::
::: {.column width="70%"}
{fig-align="center" width="100%"}
:::
:::
## Fibrosing Lung Disease in Scleroderma
When the following are present, suggest the clinical diagnosis with confidence:
<br>
::: columns
::: {.column width="32%"}
{fig-align="center" width="100%"}
<p style="text-align: center;">*anterior upper lobe* sign</p>
:::
::: {.column width="32%"}
{fig-align="center" width="85%"}
<p style="text-align: center;">*exuberant honeycombing* sign</p>
:::
::: {.column width="32%"}
{fig-align="center" width="83%"}
<p style="text-align: center;">*straight edge* sign</p>
:::
:::
## Fibrotic Hypersensitivity Pneumonitis
{fig-align="center" width="65%"}
## Typical fibrotic H.P. pattern
<br>
::: columns
::: {.column width="50%"}
{fig-align="center" width="51%"}
:::
::: {.column width="50%"}
{fig-align="center" width="45%"}
:::
:::
## Compatible (or is it Indeterminate ?) fibrotic H.P. pattern
<br>
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::: {.column width="50%"}
{fig-align="center" width="51%"}
:::
::: {.column width="50%"}
{fig-align="center" width="52%"}
:::
:::
## CT-pathologic correlation
<br>
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{fig-align="center" width="100%"}
:::
::: {.column width="70%"}
<br>
<br>
{fig-align="center" width="100%"}
:::
:::
This is the kind of case that arguably benefits from an ILD - Multidisciplinary Discussion (MDD)
## Applying the guidelines for UIP and Fibrotic Hypersensitivity Pneumonitis
It's often tougher than you might think!
{fig-align="center" width="100%"}
## Smoking-related Interstitial Fibrosis
<br>
::: columns
::: {.column width="30%"}
There are three pathologic entities worth knowing about:
* Smoking-Related Interstitial Fibrosis (SRIF)
* Smoking- Related Fibrosis with Airspace Enlargement (AEF)
* Combined Pulmonary Fibrosis-Emphysema Syndrome (CPFE)
:::
::: {.column width="70%"}
{fig-align="center" width="55%"}
:::
:::
## SRIF and Diffuse Pulmonary Hyperattenuation (GGO)
<br>
<br>
::: columns
::: {.column width="30%"}
* inter-alveolar septal fibrosis with distinctive dark-staining "ropey" collagen (arrows) on H&E staining
* produces ground glass-attenuating opacity on CT
* GGO on CT may represent both
+ smoker's macrophages and SRIF
:::
::: {.column width="70%"}
From the article:
{fig-align="center" width="100%"}
:::
:::
## An example of diffuse, background hyperattenuation abnormality and emphysema
{fig-align="center" width="100%"}
Tip: Compare the lung attenuation with air in central bronchi.
## Combined fibrosis-emphysema and airspace enlargement
<br>
::: columns
::: {.column width="30%"}
* CPFE: upper zone emphysema and bibasilar fibrosis, usually an UIP-pattern
* AEF: the cysts are often not sub-pleural and irregular in size and shape.
:::
::: {.column width="70%"}
{fig-align="center" width="45%"}
:::
:::
## Unusual causes of chronic interstitial fibrosis
<br>
::: columns
::: {.column width="30%"}
* Familial Pulmonary Fibrosis
+ definition: two or more members of the family
+ any CT pattern of fibrosis may occur, including *non-classifiable*
* Short Telomere Syndromes (Telemeropathy)
+ suspect this when premature graying, liver dysfunction, and bone marrow "failure" occur.
+ examples: Dyskeratosis Congenita and sporadic TERT (telomerase reverse transcriptase) - related mutations.
* Surfactant protein (SP)-C deficiency
+ usually presents in infancy
:::
::: {.column width="70%"}
Surfactant protein (SP)-C deficiency
{fig-align="center" width="80%"}
:::
:::
## Pleuroparenchymal Fibroelastosis
<br>
::: columns
::: {.column width="30%"}
The typical CT pattern:
* apical lung disease
* progressive, confluent reticular opacities with...
* progressive upper zone volume loss
Causes to know:
* sporadic-idiopathic
* chronic allograft dysfunction (CLAD) after lung transplantation
* may occur after stem cell transplantation
:::
::: {.column width="70%"}
{fig-align="center" width="100%"}
:::
:::
## Another case of Pleuroparenchymal Fibroelastosis
{fig-align="center" width="80%"}
Don't confuse this with insignificant Apical Caps.
## Interstitial fibrosis with non-emphysematous lung cysts
<br>
::: columns
::: {.column width="30%"}
This is very unusual. You'll get bonus points (from someone) if you suggest the diagnosis!
* SLE
* COPA syndrome (autosomal dominant)
+ named for the gene mutated in the disease, which encodes the alpha subunit of the coatomer complex-I
+ mention it if the *young* patient has unexplained arthritis and kidney disease
You'll get extra bonus points if you remember the acronym!!
:::
::: {.column width="70%"}
<br>
COPA syndrome (mutations affect a narrow amino acid stretch in the COPA gene-encoding COPα protein)
{fig-align="center" width="100%"}
:::
:::
## Interstitial Lung Abnormalities (ILAs)
<br>
::: columns
::: {.column width="30%"}
ILA is defined as *incidental* CT findings of nondependent
abnormalities affecting more than 5% of any lung zone
(ie, upper, middle, and lower lung zones are demarcated
by the levels of the inferior aortic arch and right inferior pulmonary vein) at complete or partial chest CT (eg,
abdominal or cardiac CT, including lower lung zones)
where interstitial disease was not previously suspected.
The findings include ground-glass or reticular abnormalities, lung distortion, traction bronchiectasis or bronchiolectasis, honeycombing, and nonemphysematous cysts
:::
::: {.column width="70%"}
Source:
<br>
{fig-align="center" width="85%"}
:::
:::
## What should one do if one sees this as an incidental finding ?
<br>
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{fig-align="center" width="95%"}
:::
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<br>
{fig-align="center" width="95%"}
:::
:::
## Progressive Pulmonary Fibrosis
<br>
::: columns
::: {.column width="50%"}
{fig-align="center" width="100%"}
Surveillance CT in context.
More reasonable approach: Progressive fibrosis is determined on the basis of PFTs (diminshed DLCO and FVC) and CT.
:::
::: {.column width="50%"}
{fig-align="center" width="55%"}
:::
:::
## Lymphocytic Interstitial Pneumonitis
<br>
<br>
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{fig-align="center" width="100%"}
:::
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{fig-align="center" width="75%"}
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::: {.callout-note icon="false"}
## [The best way to end an ILD talk]{style="font-size: 25px;"}
<p style="text-align: center;">Bury a disease entity!</p>
:::