Immunology of Interstitial Lung Disease, Michels, Lec Flashcards

1
Q

What are the lung defenses of upper airways and bronchi

A

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

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2
Q

host defenses in alveolar spaces

A

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

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3
Q

What are the immune cells in pulmonary immune system

A
dendritic cells, alveolar macrophages
RBC,
 lymph node dendritic cell
T cells, B cells, memory T cells
virus specific B cell
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4
Q

What is BALT and GALT

A

bronchial assoc lymphoid tissue

gut associate lymphoid tissue

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5
Q

what do macrophages look like in smoker lung

A

carbon in the macrophages

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6
Q

what does BAL look like in sarcoidosis

A

increased # lymphocytes, CD4 alveolitis

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7
Q

beaded looking cell sin BAL is indicative of what

A

asbestosis

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8
Q

Wha ttype cells will you see on BAL of scleroderma patient

A

neutrophils and eos

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9
Q

What type interstitial lung disease are corticosteroids not indicated

A

idiopathic pulmonary fibrosis

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10
Q

nonspecific interstitial pneumonia has good or poor response to corticosteroids

A

favorable

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11
Q

What events lead to development of interstitial pulmonary fibrosis

A

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

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12
Q

What is protective against radiation induced lung fibrosis

A

ACEI

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13
Q

what is evidence for cellular and humoral immunity in IPF

A

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

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14
Q

What are the genetic contributions for pulmonary fibrosis and lung cancer

A

defects in surfactant protein A2

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15
Q

genetic mutation related to familial interstitial ung disease

A

surfactant protein C gene

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16
Q

mutation in familities with idiopathic pulmonary fibrosis

A

telomerase mutations

17
Q

mutation with pulmonary fibrosis

A

MUC58 promoter polymorphism

18
Q

how does systemic sarcoidosis affect lungs

A

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

19
Q

What proteins are involved in inflammatory response in sarcoidosis

A

TNF alpha
IL7
MMP-12

20
Q

what can cause progression to persistent granulmoatous inflammation

A

persitence of Ag

Failure of immune system to halt inflammatory processes

21
Q

What is HS pneumonitis

A

group lung disease from inhalaltion of exogenous Ag molecules

22
Q

Sx HS pneumonitis

A

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

23
Q

Tx HS pnuemonitis

A

Sx resolve w/o Tx

take away exposure

24
Q

What lymphocyte count suggests HS pneumonitis

A

> 40% with higher perentage neutrophils and degranulated macrophages

25
What are the cell mediators in HS pneumonitis
TGF-b, IL-1 IL-12, TNF alpha IL-2 IFN-y IL-6 IL-17 IL-22
26
how can HS pneumonitis lead to fibrosis
genetic/environmental promoting factors that lead to alveolitis and then granulmatous inflammation leading to fibrosis
27
which T helper group is associated with chronic HS pnuemonitis vs acute
chronic TH2 | acute TH1
28
what are the major contributors in eosinophilic lung disease
lipid mediators like PAF and Leukotrience C4 chemokines cytokines major basic protein
29
whic type eosinophlic lung disease can lead to respiratory failure
acute eosinophilic pneumonia
30
BAL finding acute eos pneumonia? | blood eos count?
eos lymphocytes, neutrophils notmal blood eos count
31
CXR acute eos pneumonia
kerly B lines | diffuse opacities
32
what can cause chronic eosinophilic pneumonia
idiopathic