Pathoma - Restrictive diseases

Question 1

What is the basic premise and the lung volumes in restrictive lung disease (TLC, FEV1, FCV, FEV1:FVC)

Answer 1

Inability to FILL the lung

TLC - decreased

FEV1 - decreased

FVC - decreased

FEV1:FVC > 80% (increased) - FVC decreases more then FEV1

Question 2

What part of the lung is most commonly affected in restrictive diseases

Answer 2

Intersitium (blood-gas barrier)

Question 3

What signalling molecule is responsible for interstitial fibrosis in Idiopathic Pulmonary FIbrosis

Answer 3

TGF-b from injured pneumocytes induce fibrosis

Question 4

Describe the gross appearance of the lung in idiopathic pulmonary fibrosis

Answer 4

Honey-comb lung (fibrotic cystic changes)

Fibrosis begins in the sub-pleural space, but eventually progresses to involve the entire lung

Question 5

What cells are responsible for fibrosis in pneumoconioses

Answer 5

Chronic exposure to small particles that are engulfed by alveolar macrophages, inducing fibrosis

Question 6

Which disease is associated with shipbuilding, roofing, and plumbing

Answer 6

Asbestos

Question 7

What are the pathologic findings caused by Asbestos

Answer 7

Fibrosis of the lung or pleura (pleural plaques)

Cancer of the lung or pleura (brochogenic carcinoma > mesothelioma)

Question 8

Describe the appearance of asbestos bodies on histology

Answer 8

Golden-brown rods resembling dumbbells

Question 9

What disease is associated with miners and aerospace industry workers

Answer 9

Berylliosis

Question 10

What is the lab presentation of Berylliosis

Answer 10

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

Question 11

What disease is associated with carbon dust exposure

Answer 11

Coal workers’ pneumoconiosis

Question 12

What disease is associated with ‘black lung’

Answer 12

Coal workers’ pneumoconiosis

Question 13

What is the result of mild exposure to carbon pollution

Answer 13

Anthracosis - collection of carbon-laden macrophages

Question 14

What is the disease associated with sanblasters

Answer 14

Silicosis

Question 15

What is the pathophysiology of silicosis

Answer 15

Silica impairs phagolysosome formation by macrophages

Question 16

Which pneumoconioses occur in the upper lung and which in the lower lung?

Answer 16

Lower lung: Asbestos

Upper lung: Silicosis, Coal Workers, Berylliosis

Think:

Asbestos is from the roof but affects the base; Silica and coal are from the base (earth) but affect the roof (upper lobes)

Question 17

Which pneumoconioses is associated with increased risk for TB?

Answer 17

Silicosis (this is the desease that impairs phagolysosome formation)

Question 18

Which pneumoconioses appears similar to sarcoidosis?

Answer 18

Berylliosis

Both present as noncaseating granulomas in the lung, hilar lymph node, and systemic organs

Question 19

What is a common histologic finding in sarcoidosis?

Answer 19

Stellate inclusions (‘asteroid bodies’) within giant cells of the non-caseating granulomas

Think: sarcOID - asterOID

Question 20

What serum levels are often elevated in sarcoidosis?

Answer 20

Elevated ACE

Hypercalcemia (due to granulomas producing 1-alpha-hydroxylase, which activates vitamin D)

Think: watching a meteor shower (asterOID bodies) while eating ice cream (hypercalcemia) and playing cards (ACE)

Question 21

Treatment of sarcoidosis

Answer 21

Steroids

Question 22

What is the pathogenesis behind hypersensitivity pneumonitis

Answer 22

Mixed type III/IV hypersensitivity reaction to environmental antigen

Question 23

What antigen often causes hypersensitivity pneumonitis

Answer 23

Bird (e.g. Pigeon breeder’s lung)