Pathoma - Restrictive Diseases

Question 1

Restricted Filling of the lung causes what spirometry values to change?

Answer 1

TLC goes down (can’t fill the total lung capacity)

FVC goes way down (amount of air you can blow out after filling all the way, makes sense cause you can’t bring that much in in the first place)

FE1 goes down (amount in first second, also makes sense)

FEV1:FVC ratio is increased (opposite of airway obstruction. This makes sense because our FVC goes way down, we are reducing the denominator more than the numerator)

Question 2

Hallmark of these disorders that restrict filling

Answer 2

FEV1:FVC increases by >80% due to elastic recoil increasing

Question 3

Most common cause of restricted filling?

Answer 3

Interstitial disease

May also arise with chest abnormalities like obesity

Question 4

Interstitial fibrosis

Answer 4

Fibrosis of the elastic walls of the alveolar sac

Question 5

Idiopathic Pulmonary fibrosis

Answer 5

Fibrosis of the lung interstitium

Question 6

Histology of IPF

Answer 6

Random thickwalled, fibrotic alveolar sacs

Question 7

Etiology of IPF?

Answer 7

Related to cyclical injury of unknow origin

Question 8

Mechanism behind IPF?

Answer 8

TGF-B from injured pneumocytes induces fibrosis

Question 9

Secondary causes of IPF?

Answer 9

Drugs (Bleomycin and amioderone)

Radiation therapy

Question 10

Clinical features of IPF

Answer 10

Progressive Dyspnea and cough
Fibrosis on Lung CT
Treatment is lung transplantation

Question 11

Honeycomb lung?

Answer 11

End stage IPF

Question 12

Pneumoconioses and what causes it specifically

Answer 12

Interstitial fibrosis due to occupational exposure

Requires exposure to small particles that are fibrogenic.

The lung freaks out when it sees them and walls them off with fibrosis in an attempt to protect itself

Question 13

Exposure that leads to coal workers’ pneumoconiosis

Answer 13

Carbon dust, seen in coal miners

Question 14

Exposure that leads to silicosis

Answer 14

Silica, seen in sand blasters and silica miners

Question 15

Exposure that leads to Berylliosis

Answer 15

Beryllium - seen in berryllium miners and workers in the aerospace industry

Question 16

Exposure that leads to Asbestosis

Answer 16

• Asbestos fibers seen in construction workers, plumbers, shipyard workers

Question 17

When do we see “black lung”? Discuss it

Answer 17

In Coal Workers pneumoconiosis - Associated with rheumatoid arthritis and is a massive exposure that leads to diffuse fibrosis

Question 18

Pathologic findings for Berrylliosis?

Answer 18

Noncaseating granulomas in the lung, hilar lymph nodes and systemic organs

Question 19

____ presents with fibrosis of lungs and pleura (plaques) with increased risk for _______ and ______.

Answer 19

Asbestois
Lung Cancer
Mesothelioma

Question 20

Silicosis presents how?

Answer 20

Fibrotic nodules in upper lobes of the lung

Question 21

Besides Asbestois, this condition also increases our chance for lung cancer

Answer 21

Berrylliosis

Question 22

This condition increases our chances of TB. Why?

Answer 22

Silicosis - Silica impairs phagolysosome formation by macrophages

Question 23

Mild exposure to carbon, like in pollution, results in this condition that is not all that clinically significant

Answer 23

Anthracosis - Cllection of carbon laden macrophages

Question 24

How do we confirm exposure to asbestos?

Answer 24

Lesions may contain long, golden-brown fibers with associated iron (asbestos bodies) which confirm exposure to asbestos

Question 25

Sarcoidosis and who tends to get it?

Answer 25

Granulomas in the interstitial of the lung (noncaseating granulomas) and other organs

African American women mostly

Question 26

Cause of sarcoidosis

Answer 26

Unknown but likely due to CD4+ helper T-cell response to unknown antigen

Question 27

What does noncaseating mean?

Answer 27

No death! Nuclei all still there

Question 28

Defining histology of sarcoid granulomas?

Answer 28

Epithelioid histiocytes surrounding a giant cell granuloma that is non caseating

Question 29

Common finding in a granuloma of sarcoidosis

Answer 29

Asteroid body in the center!

Question 30

Besides lungs, what else can sarcoid hit?

Answer 30

1. Eye - Uvea - Uveitis
2. Skin - Nodules
3. Salivary or lacrimal glands (mimics sjogren’s)

Question 31

Clinical features of sarcoidosis?

Answer 31

Cough

SOB

Question 32

Labs for sarcoidosis?

Answer 32

Elevated serum ACE and hypercalcemia, caused by granulomas have 1 alpha hydroxylase activity and can activate Vitamin D (we see this in all noncaseating granulomas)

Question 33

Treatment for sarcoidosis?

Answer 33

Steroids, often resolves spontaneously without treatment

Question 34

Hypersentitivity pneumonitis

Answer 34

Patient exposed to some inhaled organic antigens (bird poop for example) that results in a granulomatous reaction

Question 35

Presentation and treatment for Hypersensitivity pneumonitis

Answer 35

Fever, cough, dyspnea hours after exposure. Resolves with removal of the exposure

Question 36

Chronic exposure of whatever is causing the hypersensitivity pneumonitis leads to what?

Answer 36

Interstitial fibrosis

Question 37

What is important to note about the granulomas involved with Hypersensitivity pneumonitis?

Answer 37

Remember this is hypersensitivity, so we will have a lot of eosinophils