Pathoma - Restrictive Diseases Flashcards Preview

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Flashcards in Pathoma - Restrictive Diseases Deck (37)
1

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

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)

2

Hallmark of these disorders that restrict filling

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

3

Most common cause of restricted filling?

Interstitial disease

May also arise with chest abnormalities like obesity

4

Interstitial fibrosis

Fibrosis of the elastic walls of the alveolar sac

5

Idiopathic Pulmonary fibrosis

Fibrosis of the lung interstitium

6

Histology of IPF

Random thickwalled, fibrotic alveolar sacs

7

Etiology of IPF?

Related to cyclical injury of unknow origin

8

Mechanism behind IPF?

TGF-B from injured pneumocytes induces fibrosis

9

Secondary causes of IPF?

Drugs (Bleomycin and amioderone)

Radiation therapy

10

Clinical features of IPF

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

11

Honeycomb lung?

End stage IPF

12

Pneumoconioses and what causes it specifically

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

13

Exposure that leads to coal workers' pneumoconiosis

Carbon dust, seen in coal miners

14

Exposure that leads to silicosis

Silica, seen in sand blasters and silica miners

15

Exposure that leads to Berylliosis

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

16

Exposure that leads to Asbestosis

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

17

When do we see "black lung"? Discuss it

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

18

Pathologic findings for Berrylliosis?

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

19

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

Asbestois
Lung Cancer
Mesothelioma

20

Silicosis presents how?

Fibrotic nodules in upper lobes of the lung

21

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

Berrylliosis

22

This condition increases our chances of TB. Why?

Silicosis - Silica impairs phagolysosome formation by macrophages

23

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

Anthracosis - Cllection of carbon laden macrophages

24

How do we confirm exposure to asbestos?

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

25

Sarcoidosis and who tends to get it?

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

African American women mostly

26

Cause of sarcoidosis

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

27

What does noncaseating mean?

No death! Nuclei all still there

28

Defining histology of sarcoid granulomas?

Epithelioid histiocytes surrounding a giant cell granuloma that is non caseating

29

Common finding in a granuloma of sarcoidosis

Asteroid body in the center!

30

Besides lungs, what else can sarcoid hit?

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

31

Clinical features of sarcoidosis?

Cough
SOB

32

Labs for sarcoidosis?

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)

33

Treatment for sarcoidosis?

Steroids, often resolves spontaneously without treatment

34

Hypersentitivity pneumonitis

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

35

Presentation and treatment for Hypersensitivity pneumonitis

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

36

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

Interstitial fibrosis

37

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

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