Restrictive Lung Disease-Bootcamp

Question 1

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Idiopathic pulmonary fibrosis is _

Answer 1

Idiopathic pulmonary fibrosis is slow onset of fibrotic deposition in the lungs of unknown etiology

Question 2

Common symptoms of IPF include:

Answer 2

Common symptoms of IPF include: dyspnea, non-productive cough, fatigue, crackles

Question 3

_ imaging is often used for the diagnosis of IPF in which _ and _ will be observed

Answer 3

CXR/CT imaging is often used for the diagnosis of IPF in which bilateral diffuse reticulonodular opacities and honeycombing will be observed
* Looks like a net-like pattern of nodular fibrosis

Question 4

Explain the pathogenesis of IPF

Answer 4

IPF involves repetitive injury, followed by disorganized repair

Question 5

The repetitive injuries that might lead to IPF include things like _ or _

Answer 5

The repetitive injuries that might lead to IPF include things like smoking or acid reflux

Question 6

Explain how the repair process of the lungs becomes disorganized in IPF

Answer 6

Type 1 pneumocytes are injured + abnormal BM –>
Type 2 pneumocytes will try to regenerate them –> Hyperplasia with failed differentiation (due to abnormal BM) –>
Impaired re-epithelialization causes fibroblasts to undergo focal proliferation and lay down collagen –>
Fibrotic lungs

Question 7

When a known chemical or radiation causes damage to the lung resulting in scarring and fibrosis, we call this _

Answer 7

When a known chemical or radiation causes damage to the lung resulting in scarring and fibrosis, we call this drugs and radiation-induced fibrosis
* This is not considered IPF because it has known cause

Question 8

Four drugs commonly associated with lung fibrosis

Answer 8

1. Bleomycin
2. Amiodarone
3. Methotrexate
4. Busulfan

Question 9

For the first 12 weeks post radiation the effect on the lung is _

Answer 9

For the first 12 weeks post radiation the effect on the lung is neumonitis (inflammation)

Question 10

Fibrosis can occur _ months after radiation

Answer 10

Fibrosis can occur 6-12 months after radiation

Question 11

In order for Type II pneumocytes to regenerate Type I pneumocytes there must be presense of _

Answer 11

In order for Type II pneumocytes to regenerate Type I pneumocytes there must be presense of normal basement membrane

Question 12

What is pneumoconiosis?

Answer 12

Pneumoconiosis is an occupationally acquired restrictive lung disease that occurs after many years of inhaling dust particles

Question 13

Pneumoconiosis pathogenesis begins with dust particles inhaled –> ?

Answer 13

Dust particles inhaled into alveoli –> Alveolar macrophages phagocytose dust –> Trigger cytokine release –> Activation of fibroblasts –> Fibrosis

Question 14

Blackened “carbon-laden” macrophages in the upper lungs is called _ and is associated with _

Answer 14

Blackened “carbon-laden” macrophages in the upper lungs is called anthracosis and is associated with coal-miner’s pneumoconiosis

Question 15

_ pneumoconiosis is associated with an increased risk of TB infection

Answer 15

Silicosis is associated with an increased risk of TB infection
* Silicosis causes macrophage dysfunction

Question 16

The most common exposure to silicosis is through occupations like _ or _

Answer 16

The most common exposure to silicosis is through occupations like sandblasting or working in foundries

Question 17

The classic CXR finding associated with silicosis is _

Answer 17

The classic CXR finding associated with silicosis is eggshell calcifications in the hilar lymph nodes

Question 18

Berylliosis is associated with occupations like _

Answer 18

Berylliosis is associated with occupations like aerospace manufacturing

Question 19

Inhalation of beryllium is associated with _ pathology

Answer 19

Inhalation of beryllium is associated with formation of non-caseating granulomas and enlarged lymph nodes
* Also associated with increased lung cancer risk

Question 20

Asbestosis is most common in individuals with a history of _

Answer 20

Asbestosis is most common in individuals with a history of roofing, shipbuilding, house insulation, plumbing

Question 21

The effect of asbestos on the lung interstitium is _

Answer 21

The effect of asbestos on the lung interstitium is lower lung lobe fibrosis and bronchogenic carcinoma

Question 21

Over time, the asbestos fibers sitting in the lung interstitium/ pleura can get coated in iron and form _ bodies

Answer 21

Over time, the asbestos fibers sitting in the lung interstitium/ pleura can get coated in iron and form ferruginous bodies
* These are “golden fusiform iron-coated fibers found in asbestos lesions”

Question 21

The effect of asbestos on the lung pleura is _

Answer 21

The effect of asbestos on the lung pleura is
* Calcified pleural plaques
* Pleural effusions
* Mesothelioma (parietal pleura)

Question 22

The most common cancer risk for asbestosis patients is _

Answer 22

The most common cancer risk for asbestosis patients is bronchogenic carcinoma
* This is more common than mesothelioma in this population

Question 23

_ causes of pneumoconiosis tend to affect the upper lungs while _ affects the lower lungs

Answer 23

Coal miners, silicosis, berylliosis tend to affect the upper lungs while asbestosis affects the lower lungs

Question 24

What is hypersensitivity pneumonitis?

Answer 24

Hypersensitivity pneumonitis is a hypersensitivity reaction to environmental antigens that causes cough, granuloma formation, and eventual fibrosis

Question 25

Hypersensitivity pneumonitis is a type _ hypersensitivity reaction

Answer 25

Hypersensitivity pneumonitis is a Type III/IV hypersensitivity reaction

Question 26

Common environmental antigens that can cause hypersensitivity pneumonitis include _

Answer 26

Common environmental antigens that can cause hypersensitivity pneumonitis include mold, bacteria, agricultural particles

Question 27

Acutely, hypersensitivity pneumonitis causes _

Answer 27

Acutely, hypersensitivity pneumonitis causes inflammation that causes dyspnea, cough, fever

Question 28

Chronically, hypersensitivity pneumonitis can cause _

Answer 28

Chronically, hypersensitivity pneumonitis can cause non-caseating granuloma formation and fibrosis
* Therefore, people are advised to avoid exposure to the antigen

Question 29

Two classic cases of hypersensitivity pneumonitis are _ and _

Answer 29

Two classic cases of hypersensitivity pneumonitis are farmers breathing moldy hay and bird-fanciers breathing bird proteins

Question 30

We can diagnose hypersensitivity pneumonitis by performing _ procedure which reveals _

Answer 30

We can diagnose hypersensitivity pneumonitis by performing bronchoalveolar lavage which reveals leukocytes