9.5 Restrictive Diseases

Question 1

What happens to the TLC in restrictive diseases?

Answer 1

Decreases

Question 2

What happens to the FEV1/FVC in restrictive diseases?

Answer 2

Increased (greater than 80%)

Question 3

What happens to the FVC in restrictive diseases?

Answer 3

Decreases drastically

Question 4

What happens to the FEV1 in restrictive diseases?

Answer 4

Decreases slightly

Question 5

What is the most common cause of restrictive diseases?

Answer 5

Interstitial Disease

Question 6

What are some other causes of restrictive diseases?

Answer 6

Chest Wall Disease

Question 7

Idiopathic Pulmonary Fibrosis

Answer 7

Fibrosis of lung interstitium

Question 8

What is the pathogenesis of IPF?

Answer 8

It is due to cyclical lung injury with TGF-b from injured pneumocytes will induce the fibrosis

Question 9

What are some drugs that can cause Interstitial fibrosis

Answer 9

Amiodarone

Bleomycin

Question 10

Where does the fibrosis from IPF start at?

Answer 10

Sub-pleural space

Question 11

Pneumoconiosis

Answer 11

Interstitial fibrosis due to exposure to different types of dust that are fibrogenic

Question 12

What is fibrosis in pneumoconiosis caused by?

Answer 12

Macrophages

Question 13

Coal Worker’s Pneumoconiosis

Answer 13

Carbon is taken in from coal miners most often that results in a black lung

Question 14

Caplan Syndrome

Answer 14

Rheumatoid Arthritis associated with coal worker’s pneumoconiosis

Question 15

Anthracosis

Answer 15

Collections of carbon laden macrophages in the lungs

Question 16

Silicosis

Answer 16

Silica is taken in often in sand blasters and it impairs the formation of the phagolysosome in macrophages - the only pneumoconiosis that increases risk for TB

Question 17

Where are the fibrotic nodules from silicosis found?

Answer 17

Upper lobes of the lung

Question 18

Berylliosis

Answer 18

Beryllium in miners or aerospace workers that cause noncaseating granulomas in the lungs with increased risk for lung cancer - looks like sarcoidosis

Question 19

Asbestosis

Answer 19

Asbestos in construction, plumbers or ship builders which causes fibrosis of the lung and pleura as well as carcinoma of the lung and mesothelioma

Question 20

What will be seen on histology of asbestosis?

Answer 20

Ferruginous Bodies

Question 21

Sarcoidosis

Answer 21

Systemic disease characterized with noncaseating granulomas in multiple organs

Question 22

Who is the classical patient of sarcoidosis?

Answer 22

African American females

Question 23

What are the characteristic findings of the granulomas in sarcoidosis?

Answer 23

• Asteroid Body

- Schaumann Body

Question 24

What are the clinical features of sarcoidosis?

Answer 24

• Dyspnea
• Cough
• Elevated serum ACE
• Hypercalcemia - excessive 1-alpha hydroxylase activity which produces more Ca2+

Question 25

Hypersensitivity Pneumonitis

Answer 25

Granulomatous reaction to inhaled organic antigens and resolves with removal from the exposure - eosinophils will be seen

Question 26

What happens with chronic hypersensitivity pneumonitis?

Answer 26

It will lead to interstitial fibrosis