Restrictive Diseases - Pathoma

Question 1

What is the main problem in restrictive lung diseases?

Answer 1

restricted filling of the lung=

Question 2

What are the PFT’s in restrictive lung disease?

Answer 2

• decreased TLC
• significantly decreased FVC, moderately decreased FEV1
• FEV1:FVC ratio is increased (>80%)

Question 3

What are restrictive diseases most commonly due to?

Answer 3

Interstitial disease of the lung => fibrosis of the interstitium
(may also arise with chest wall abnormalities, e.g. obesity)

Question 4

What is the interstitium of the lung?

Answer 4

Wall of the alveolar air sacs or gas barrier

Question 5

What happens is Idiopathic Pulmonary Fibrosis?

Answer 5

fibrosis of lung interstitium

Question 6

What is the etiology of Idiopathic Pulmonary Fibrosis related to?

Answer 6

Cyclical lung injury:

-TGF-beta from injured pneumocytes induces fibrosis (induces cyclical healing)

Question 7

When diagnosing a patient with Idiopathic Pulmonary Fibrosis, what conditions must be exclued?

Answer 7

Other causes:

Secondary causes such as drugs (e.g. bleomycin and amiodarone) and radiation therapy

Question 8

What are the clinical features of Idiopathic Pulmonary Fibrosis?

Answer 8

• Progressive dyspnea and cough
• Fibrosis on lung CT (entire lung => “honeycomb lung”)
• Treatment is lung transplantation

Question 9

What are Pneumoconioses?

Answer 9

Disorders in which you get interstitial fibrosis due to some occupational exposure
=>activates alveolar macrophages to lay down fibrous tissue

Question 10

What kind of exposure is required to develop Pneumoconioses?

Answer 10

Requires chronic exposure to small particles that are fibrogenic => get to bottom of the lung & induce fibrosis (mucus filters big particles out)

Question 11

What are the four most common types of Pneumoconioses?

Answer 11

1. Coal Workers’ Pneumoconioses
2. Silicosis
3. Berylliosis
4. Asbestosis

Question 12

What is the small particle in Coal Workers’ Pneumoconioses that induces fibrosis?

Answer 12

carbon dust

Question 13

What pathological findings are seen in lungs with Coal Workers’ Pneumoconioses?

Answer 13

Diffuse fibrosis = “Black Lung”

Shrunken lungs

Question 14

What joint disease is associated with Coal Workers’ Pneumoconioses?

Answer 14

Rhematoid Arthritis => Caplan Syndrome

Question 15

What do you call carbon build up in alveolar macrophages due to mild carbon exposure?

Answer 15

Anthracosis

collection of carbon-laden macrophages

Question 16

What kind of exposure causes Silicosis?

Answer 16

SILICA

-seen in sandblasters and silica miners

Question 17

What pathological findings are seen in lungs with Silicosis?

Answer 17

***Impairs phagolysosomes formation by macrophages => causes fibrotic nodules in UPPER lobes of the lungs

Question 18

What is the only type of Pneumoconioses that increases the risk for TB?

Answer 18

Silicosis

Question 19

What type of occupation do you see Berylliosis?

Answer 19

• Beryllium miners

- Aerospace industry

Question 20

What pathological findings are seen in Berylliosis?

Answer 20

Noncaseating granulomas in the lung, hilar lymph nodes, and systemic organs (similar to sarcoidosis)

Question 21

Patient’s with Berylliosis have an increased risk for what condition?

Answer 21

Lung cancer :(

Question 22

What type of occupations are exposed to asbestos?

Answer 22

• construction workers
• plumbers
• shipyard workers

Question 23

What are the common pathological findings seen in Asbestosis?

Answer 23

Fibrosis of lung and pleura (plaques) with increased risk for lung carcinoma and mesothelioma
***Lung carcinoma is more common than mesothelioma (cancer of the pleura) in exposed individuals!

Question 24

How do you confirm exposure to asbestos or Asbestosis?

Answer 24

lesions containing long, golden-brown fibers with associated iron => “asbestos bodies”

Question 25

How does Sarcoidosis cause restrictive filling of the lung?

Answer 25

noncaseating granulomatous deposits in the interstitium of the lung => results in restricted filling

Question 26

What is the etiology of Sarcoidosis?

Answer 26

Unknown:

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

Question 27

What race/gender is most commonly affected by Sarcoidosis?

Answer 27

Classically seen in African American females

Question 28

What is the defining cell of a granuloma?

Answer 28

epitheliod histiocyte

Question 29

What unique cell is found in Sarcoid granulomas?

Answer 29

Asteroid body

funny configuration of giant cells

Question 30

Where do granulomas most commonly form in Sarcoidosis?

Answer 30

hilar lymph nodes & the lungs => leads to restrictive disease (less compliant)

Question 31

What is the typical presentation in Sarcoidosis?

Answer 31

• Cough or dyspnea
• Elevated serum ACE
• Hypercalcemia (why? granulomas activate Vitamin D)

Question 32

What causes Hypersensitivity Pneumonitis?

Answer 32

inhale some organic antigen (e.g. feces of bird/fethers) => granulomatous reaction to antigen => EOSINOPHILS

Question 33

What initial symptoms do patients with Hypersensitivity Pneumonitis present with?

Answer 33

Hours after exposure:

• Fever
• Cough
• Dyspnea

***Resolves with removal of the exposure

Question 34

What chronic symptoms do patients with Hypersensitivity Pneumonitis present with?

Answer 34

Interstitial fibrosis => restrictive lung disease