Restrictive Lung Disease

Question 1

What are the major Sx’s of restrictive lung diseases?

Answer 1

Dyspnea, Tachypnea
End-inspiratory (velcro) crackles
Cyanosis

Question 2

What sorts of things would be seen in CXR?

Answer 2

B/l nodules
Kerley B lines
Ground-glass shadows

Question 3

What severe disease are associated/caused by restrictive lung diseases?

Answer 3

Pulmonary HTN
Cor pulmonale
R-sided HF

Question 4

What is the origination of the pathogenesis of Idiopathic Pulmonary Fibrosis?

Answer 4

Aberrant repair of recurrent alveolar epithelial cell injuries caused by environmental exposures

Question 5

Mutations in which genes are associated with IPF?

Answer 5

TERT

TERC

Question 6

What is the age range for IPF and what is the prognosis with no Tx?

Answer 6

55-75 yo

3 years

Question 7

What is the only curative Tx?

Answer 7

Transplant

Question 8

If a female, non-smoker around 60 yo presents with b/l, symmetric, lower lobe opacities on radiograph and dyspnea and cough, what is your Dx?

Answer 8

Non-specific Interstitial Pneumonia

Question 9

What is the pathogenesis of Pneumoconiosis?

Answer 9

Macrophages engulf inhaled particles –> activation of innate immune cells –> lung destruction

Question 10

What are the severe complications of Coal Workers’ Pneumoconiosis?

Answer 10

Pulmonary HTN

Cor pulmonale

Question 11

Which lobes does CWP mostly affect?

Answer 11

Upper lobes

Question 12

Does CWP increase risk of cancer?

Answer 12

No

Question 13

Which population is at an increased risk for Silicosis?

Answer 13

AA

Question 14

What is the pathogenesis of Silicosis?

Answer 14

Macrophages engulf inhaled particles –> inflammasome activation –> release of IL-1/IL-18 proinflammatory mediators

Question 15

Pts with Silicosis have an increased risk for which two pathologies?

Answer 15

TB

Lung cancer

Question 16

Which lobes does Silicosis mostly affect?

Answer 16

Upper lobes

Question 17

Which form of asbestos is more virulent?

Answer 17

Serpentine/Chrysotile

Question 18

What is the pathogenesis of Asbestos-related pneumoconiosis?

Answer 18

Macrophages engulf inhaled particles –> inflammasome activation –> release of IL-1/IL-18 proinflammatory mediators

Question 19

What is seen on CXR in pts with Asbestos-related pneumoconiosis?

Answer 19

Irregular linear densities in b/l lower lobes with pleural plaques

Question 20

In which areas is Sarcoidosis most likely to present?

Answer 20

B/l hilar LNs
Lung
Eyes
Skin

Question 21

Which populations are most susceptible?

Answer 21

Women < 40 yo
10x AA
SE U.S.

Question 22

Which cells predominate in the pathogenesis of Sarcoidosis?

Answer 22

CD4 T cells

Question 23

What is the pathogenesis of Hypersensitivity Pneumonitis?

Answer 23

Immunologically mediated interstitial lung disease due to prolonged exposure to inhaled Ags

Question 24

Which type of Hypersensitivity Pneumonitis is due to dust from warm, newly harvested hay with thermophilic spores?

Answer 24

Farmers Pneumonitis

Question 25

Which type of Hypersensitivity Pneumonitis is due to proteins from serum, excreta, or feathers of birds?

Answer 25

Pigeon Breeder's Pneumonitis

Question 26

Which type of Hypersensitivity Pneumonitis is due to thermophilic bacteria in heated water reservoirs?

Answer 26

Humidifier/AC Pneumonitis

Question 27

Non-caseating granulomas suggesting a T cell-mediated type 4 rxn

Answer 27

Hypersensitivity Pneumonitis

Question 28

What are the major Sx's and CXR findings of Hypersensitivity Pneumonitis?

Answer 28

Fever, dyspnea, cough, leukocytosis | Micronodular interstitial infiltrates

Question 29

Pulmonary eosinophilia is characterized by elevated alveolar levels of ___

Answer 29

IL-5 (eosinophil attractant)

Question 30

Dyspnea, dry cough, and clubbing in a 40-50 yo smoker with presence of Smokers' Macrophages is characteristic of what disease?

Answer 30

Desquamative Interstitial Pneumonia

Question 31

What type of pt will characteristically have Pulmonary Langerhans Cell Histiocytosis?

Answer 31

Young smokers (get better with cessation)

Question 32

Accumulation of surfactant in alveoli due to GM-CSF dysfunction is characteristic of what pathology?

Answer 32

Pulmonary Alveolar Proteinosis

Question 33

What will be seen on CXR for Pulmonary Alveolar Proteinosis?

Answer 33

B/l patchy, asymmetrical pulmonary opacifications

Question 34

What is the characteristic Sx of adults with Pulmonary Alveolar Proteinosis?

Answer 34

Chunky, gelatinous sputum

Question 35

AR inheritance of the ABCA3 gene leading to dysfunction of surfactant trafficking/secretion is characteristic of what pathology?

Answer 35

Surfactant Dysfunction Disorder