Pathology of interstitial lung disease

Question 1

what is the hallmark of chronic interstitial disease?

Answer 1

reduced compliance (stiff lungs) leading to dyspnea and hypoxia

Question 2

honeycomb lung is the end stage of what condition?

Answer 2

lung disease

Question 3

what is the end stage of lung disease?

Answer 3

honeycomb lung

Question 4

ARDS corresponds to a severe form of what condition?

Answer 4

acute lung injury

Question 5

what is the first event in acute lung injury?

Answer 5

endothelial damage

Question 6

what is the general cause of ARDS? what is a specific condition that can cause it?

Answer 6

diffuse alveolar capillary damage (endothelial damage then epithelial damage)

DAD (diffuse alveolar damage)

Question 7

what is the most common cause of non-cardiogenic pulmonary edema?

Answer 7

ARDS

Question 8

what are the most common direct injuries related to ARDS?

Answer 8

infectious agents (pneumonia) 
aspiration

Question 9

what are the most common indirect injuries related to ARDS?

Answer 9

shock

sepsis

Question 10

what are the characteristics of ARDS? (CXR, wedge pressure)

Answer 10

CXR - bilateral infiltrates

wedge pressure - less than 18 mm Hg

Question 11

what are the paO2s for ALI and ARDS?

Answer 11

ALI - less than 300

ARDS - less than 200

Question 12

what is the pathogenesis of ARDS?

Answer 12

imbalance between proinflammatory and anti-inflammatory mediators
increased vascular permeability
loss of diffusion capacity
widespread surfactant abnormalities

Question 13

IL-8 is a potent chemoattractant for what cell type?

Answer 13

neutrophils

Question 14

what chemoattractant attracts neutrophils?

Answer 14

IL-8

Question 15

what is the gross morphology of the lungs in ARDS?

Answer 15

heavy, firm, red, stiff

Question 16

what is the micro morphology of the lungs in ARDS?

Answer 16

alveolar hyaline membranes

proliferation of type II pneumocytes

Question 17

TRALI is due to what type of antibodies?

Answer 17

anti-HLA or anti-HNA

Question 18

diffuse interstitial disease initially starts out as what disease?

Answer 18

alveolitis

Question 19

what is the final stage of diffuse interstitial disease?

Answer 19

end stage fibrotic lung (honeycomb lung)

Question 20

what is the histological pattern of UIP?

Answer 20

fibrosis

Question 21

what cell type plays a key role in inflammation seen in diffuse interstitial disease?

Answer 21

macrophage

Question 22

is UIP specific for IPF?

Answer 22

no

Question 23

what is the pathogenesis of IPF? what cell types / molecules are involved?

Answer 23

repeated cycles of epithelial activation / injury by some unidentified agent - abnormal wound healing

Th2 T cells, TGFB-1

Question 24

what molecules blocks the action of caveolin in fibroblasts? what is the result?

Answer 24

TGFB-1

overabundance of fibrosis

Question 25

what is the microscopic appearance of IPF?

Answer 25

temporal heterogeneity - early and late lesions

Question 26

what is the hallmark appearance of UIP?

Answer 26

patchy interstitial fibrosis, varying in intensity and age

Question 27

which lung diseases require transplant?

Answer 27

UIP (IPF)

Question 28

definition: pneumoconioses

Answer 28

non-neoplastic lung reaction to inhalation of any aerosol (including air pollution)

Question 29

what is the key factor in pneumoconioses pathogenesis?

Answer 29

capacity of inhaled dusts to stimulate fibrosis