Immunology of Interstitial Lung Disease, Michels, Lec

Question 1

What are the lung defenses of upper airways and bronchi

Answer 1

anatomic barriers, cough, mucociliary apparatus, airway epithelium
secretory IgA
dendritic cells, lymphocytes and Neutrophils

Question 2

host defenses in alveolar spaces

Answer 2

alveolar macrophages
Ig, opsonins, surfactants
lymophocyte-mediated immunity
Neutrophils and eosinophils

Question 3

What are the immune cells in pulmonary immune system

Answer 3

dendritic cells, alveolar macrophages
RBC,
 lymph node dendritic cell
T cells, B cells, memory T cells
virus specific B cell

Question 4

What is BALT and GALT

Answer 4

bronchial assoc lymphoid tissue

gut associate lymphoid tissue

Question 5

what do macrophages look like in smoker lung

Answer 5

carbon in the macrophages

Question 6

what does BAL look like in sarcoidosis

Answer 6

increased # lymphocytes, CD4 alveolitis

Question 7

beaded looking cell sin BAL is indicative of what

Answer 7

asbestosis

Question 8

Wha ttype cells will you see on BAL of scleroderma patient

Answer 8

neutrophils and eos

Question 9

What type interstitial lung disease are corticosteroids not indicated

Answer 9

idiopathic pulmonary fibrosis

Question 10

nonspecific interstitial pneumonia has good or poor response to corticosteroids

Answer 10

favorable

Question 11

What events lead to development of interstitial pulmonary fibrosis

Answer 11

vasodialtion, coagulation, oxidative stress, vascualr remodeling
TGF-beta!!!! from alveolar macrophages
TNF alpha
PDGF

Question 12

What is protective against radiation induced lung fibrosis

Answer 12

ACEI

Question 13

what is evidence for cellular and humoral immunity in IPF

Answer 13

CD4 T cells
biased T cell R Vbeta
presence of autoAb in some studies
lymphoid neogenesis without organization

Question 14

What are the genetic contributions for pulmonary fibrosis and lung cancer

Answer 14

defects in surfactant protein A2

Question 15

genetic mutation related to familial interstitial ung disease

Answer 15

surfactant protein C gene

Question 16

mutation in familities with idiopathic pulmonary fibrosis

Answer 16

telomerase mutations

Question 17

mutation with pulmonary fibrosis

Answer 17

MUC58 promoter polymorphism

Question 18

how does systemic sarcoidosis affect lungs

Answer 18

interstitial pneumonitis and granulmatous formation progress to fibrosis
damage alveolar and bronchial tissue and vascular surface area

Question 19

What proteins are involved in inflammatory response in sarcoidosis

Answer 19

TNF alpha
IL7
MMP-12

Question 20

what can cause progression to persistent granulmoatous inflammation

Answer 20

persitence of Ag

Failure of immune system to halt inflammatory processes

Question 21

What is HS pneumonitis

Answer 21

group lung disease from inhalaltion of exogenous Ag molecules

Question 22

Sx HS pneumonitis

Answer 22

transient fever, hypoxemia, myalgias, arthralgias, dyspnea, cough 2-9 hrs post exposure

Question 23

Tx HS pnuemonitis

Answer 23

Sx resolve w/o Tx

take away exposure

Question 24

What lymphocyte count suggests HS pneumonitis

Answer 24

> 40% with higher perentage neutrophils and degranulated macrophages

Question 25

What are the cell mediators in HS pneumonitis

Answer 25

TGF-b, IL-1 IL-12, TNF alpha
IL-2 IFN-y
IL-6 IL-17 IL-22

Question 26

how can HS pneumonitis lead to fibrosis

Answer 26

genetic/environmental promoting factors that lead to alveolitis and then granulmatous inflammation leading to fibrosis

Question 27

which T helper group is associated with chronic HS pnuemonitis vs acute

Answer 27

chronic TH2

acute TH1

Question 28

what are the major contributors in eosinophilic lung disease

Answer 28

lipid mediators like PAF and Leukotrience C4
chemokines
cytokines
major basic protein

Question 29

whic type eosinophlic lung disease can lead to respiratory failure

Answer 29

acute eosinophilic pneumonia

Question 30

BAL finding acute eos pneumonia?

blood eos count?

Answer 30

eos
lymphocytes, neutrophils
notmal blood eos count

Question 31

CXR acute eos pneumonia

Answer 31

kerly B lines

diffuse opacities

Question 32

what can cause chronic eosinophilic pneumonia

Answer 32

idiopathic