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Block 9 Week 3 > Restrictive Lung Disease - Parks and Baker > Flashcards

Restrictive Lung Disease - Parks and Baker Flashcards

1
Q 
what are the following values in Restrictive lung disease
TLC
DLCO
FVC
FEV1
A

dec. TLC
dec. DLCO
dec. FVC
normal/inc FEV1

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2
Q

what are the chest wall mediated causes of RLD?

A
  1. neuromuscular disorders
  2. obesity
  3. pleural disorders
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3
Q

what are the fibrosing causes of RLD?

A
  1. idiopathic pulmonary fibrosis
  2. non-specific intersitial pneumonia
  3. cytogenic organizing pneumonia
  4. connective tissue disroders
  5. pneumoconiosis
  6. drug rxns
  7. radiation pneumonitis
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4
Q

what two autoimmune dz can cause fibrosis of the lung?

A

RA

SLE

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5
Q

describe the process of forming a honeycomb lung.

A
  1. lung is injured
  2. lung reacts with inflammation and wound healing
  3. the distal airway ends up being “amputated” by the fibrosis and becomes a dead air space not connected to anything
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6
Q

what type of inflammation causes honeycombing?

A

interstitial and alveolar inflammation

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7
Q

What are the three general categories of things needed to Dx IPF?

A
  1. clinical picture
  2. CXR
  3. pathologic changes
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8
Q

What are the pathologic changes in IPF known as?

A

usual intersitial pneumonia UIP

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9
Q

What other diseases show UIP?

A

connective tissue disorders
chronic hypersensitivity reactions
pneumonia
asbestosis

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10
Q

You need to have (UIP/IPF), but you don’t necessarily have (UIP/IPF) if you have (UIP/IPF)

A

you need UIP to have IPF; but having UIP doesn’t mean you automatically have IPF

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11
Q

which lobe of the lung is most affected by IPF?

A

lower lobes; most fibrosed

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12
Q

Honeycombing is also called what pathologically?

A

cystic spaces within fibrosis

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13
Q

how soon does IPF kill you?

A

3-5 yyears

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14
Q

What kind of fibrosis takes place in IPF?

A

subpleural

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15
Q

histologically, what are the earliest lesions seen in IPF?

A

fibroblastic foci

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16
Q

What are the three histologic findings that are characteristic for IPf?

A
  1. fibroblastic focus
  2. subpleural fibrosis
  3. temporal heterogeneity
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17
Q

Subpleural fibrosis leads to…

A

collapse and obliteration of alveolar air spaces

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18
Q

What is the MOA hypothesis for the formation of IPF?

A
  1. repeated stimulus
  2. sequential lung injury
  3. ABERRANT WOUND HEALING
    a. inflmmation/Th1/2balance / genetics
  4. fibrosis
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19
Q

What are the environmental factors that lead to IPF?

A
  1. smoking
  2. exposure to metal fumes and wood dust
  3. hair dressers
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20
Q

the genetic predispositions that affect IPF are associated with what cell type?

A

pneumocytes

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21
Q

What age is associated with IPF?

A

> 50

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22
Q

Nintedanib inhibits (blank) for growth factors for fibroblasts in IPF

A

tyrosine kinase receptors

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23
Q

how does IPF first present?

A

dry cough and dyspnea

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24
Q

when does IPF normally onset?

A

40-70

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25
Q

when does clubbing occur in IPF?

A

late; indicates hypoxemia

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26
Q

What are the Tx available for IPF?

A

steroids
immunosuppressants
lung transplant

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27
Q

what is the big difference between NSIP (nonspecific interstitial pneumonia) and IPF?

A

NSIP Biopsies fail to show diagnostic features of any of the other well-characterized ILDs
aka
looks like IPF, but LACKS UIP PATTERN

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28
Q

what are the two types of NSIP?

A

cellular

fibrosing

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29
Q

which type of NSIP happens in younger people and has better outcomes?

A

cellular

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30
Q

which NSIP has NO temporal heterogeneity or honeycombing

A

fibrosing

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31
Q

which NSIP is characterized by moderate chronic interstitial INFLAMMATION

A

cellular

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32
Q

which NSIP is characterized by diffuse or pathy FIBROSIS

A

fibrosing

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33
Q

When UIP and NSIP are both present, prognosis is only as good as the (blank) lesion

A

worst

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34
Q

how does NSIP present?

A

40-55; dyspnea with cough for a few months

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35
Q

How does Cryptogenic organizing pneumonia (BOOP) present?

A

cough with dypsnea

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36
Q

what do you see on CXR in BOOP

A

pathy airspace opacities

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37
Q

what does the organizing mean in cryptogenic organizing pneumonia?

A

long term changes of fibrinous exudates

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38
Q

Polyploid plugs of loose organizing connective tissue, aka (blank), are seen in BOOP

A

Masson bodies

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39
Q

does BOOP present with temporal hetergeneity, honeycombing, or interstitial fibrosis?

A

nope

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40
Q

T/F: the lung architecture in BOOP is normal

A

true

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41
Q

Masson bodies are massive of what type of tissue?

A

granulation tissue

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42
Q

what is the reason that Masson bodies form?

A

When you get a regular strep pneumo, you have to heal.

But these people come in with a cryptogenic (aka idiopathic) pneumonia that forms granulation tissue to heal

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43
Q

Organizing pneumonia with intra-alveolar fibrosis can be secondary to….

A 
infections
inflamm/injury
inhaled toxins
drugs
connective tissue disease
GVHD
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44
Q

what should your history really check for with organizing pneumonia

A

workplace exposure to chemicals
arthralgias
malar rash
RA or SLE

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45
Q

Describe the lung involvement in RA?

A
  1. chronic pleuritis
  2. diffuse interstitial pneumonitis and fibrosis
  3. intrapulm. rheumatoid nodules
  4. pHTN
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46
Q

Describe the lung involvement in systemic sclerosis?

A

diffuse interstitial fibrosis; UIP or NSIP

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47
Q

describe the lung involvement in lupus?

A

patchy, transient parenchymal infiltrates

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48
Q

what are pneumoconioses?

A

Diseases induced by inhalation of organic and inorganic particulates, chemical fumes and vapors.

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49
Q

what particle size is most dangerous to the lungs?

A

1-5um; settles in distal airways

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50
Q

Small particles can reach toxic levels and cause acute lung injury while large particles stay in the lung parenchyma and evoke what ?

A

fibrosing collagenous pneumoconioses

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51
Q

T/F: pneumoconioses cannot travel by blood or lymph

A

false!

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52
Q

Describe the pathogenesis of injury in pneumoconioses?

A
  1. Dust activates mac’s
  2. mac’s release IL1, TNF-a, and ROS
  3. mediators lead to continual cell injury
  4. Fibroblast proliferation, collagen deposition
  5. interstitial fibrosis
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53
Q

what color do the lymph nodes become with continual particulate exposure?

A

anthracotic (black)

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54
Q

describe a silicotic nodule

A

collagen core surrounded by fibroblasts and lymphs

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55
Q

describe an asbestotic alveolus

A

asbestos bodies within the alveolus with interstitial fibrosis

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56
Q

which pleura becomes thickened in asbestosis?

A

visceral pleura

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57
Q

which lobes of the lung are most affected in asbestosis?

A

lower

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58
Q

smoking reduces (blank) clearance

A

mucociliary

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59
Q

Smoking worsens the effects of all inhaled dusts, but particularly…

A

asbestos

60
Q

What are the three severities of coal worker’s lung?

A
  1. anthracosis
  2. simple CWP
  3. complicated CWP
61
Q

t/f; CWP has little to no decrease in lung function

A

true

62
Q

a minority of patients with CWP will progress to..

A

progressive massive fibrosis (PMF)

63
Q

T/F: coal worker’s lung has no increased risk of TB or cancer

A

true

64
Q

CWP is associated with what other lung diseases?

A

COPD

65
Q

what is the most common occupational disease in the world?

A

silicosis

66
Q

Describe the lesions in silicosis?

A

nodular and fibrosing; seen in progressive exposure

67
Q

what do you see in actue silicosis?

A

Abundant lipoproteinaceous material in alveoli

68
Q

actue silicosis is identical to what other disease?

A

alveolar proteinosis

69
Q

what element causes silicosis?

A

quartz

70
Q

T/F: pure quartz is worse for your lungs than quartz mixed with other shit

A

true

71
Q

Early in silicosis, the tiny nodules will later become…

A

hard collagenous scars

72
Q

what technique will show silica crystals in a biopsy?

A

polarized microscopy

73
Q

which lobes of the lung are most affected by silicosis?

A

upper zone nodularity

74
Q

When do you get SOB in silicosis

A

late, when you get massive fibrosis

75
Q

Describe the hilar calcification in silicosis

A

EGGSHELL calcification

76
Q

T/F: silicosis makes you more susceptible to TB

A

true

77
Q

T/F: silicosis makes you more susceptible to cancer

A

true; possibly carcinogenic

78
Q

What are the effects on the pleura of asbestos?

A

Pleural Effusions
Fibrous plaques
Diffuse pleural fibrosis
Mesothelioma

79
Q

what are the effects on the lung parenchyma of asbestos?

A

lung carcinoma

interstitial fibrosis aka Asbestosis

80
Q

mesothelioma is cancer of the…

A

pleura

81
Q

what is the most common type of asbestos used in industry?

A

serpentine (curly)

82
Q

why is serpentine asbestos less likely to cause pathology?

A

gets stuck higher up in the lung

83
Q

what is the only type of asbestos associated with mesothelioma?

A

amphibole (needle like)

84
Q

T/F: smoking increases the risk of having mesothelioma

A

FALSE

85
Q

What is asbestosis in pathology terms?

A

Diffuse pulmonary interstitial fibrosis

86
Q

how to asbestos bodies form?

A

macs try to phagocytize the fibers

87
Q

what is another name for asbestos bodies?

A

feruginous bodies: aka coated in iron (Fe)

88
Q

asbestosis has a similar appearance histologically to…

A

UIP

89
Q

What parts of the lung are fist affected in asbestosis?

A

lower lung and subpleura areas

90
Q

Describe the presentation of asbestosis?

A

DOE first, then Dyspnea with rest, productive cough

91
Q

How common is asbestosis after:
10 years exposure
>20 years exposure

A

rare at 10

common at 20 plus

92
Q

what does the CXR look like in asbestosis?

A

fine reticular pattern with greater dominance in the lower lobes

93
Q

Pleural plaques from asbestos are well circumscribed and dense with what two things?

A

collagen and Ca

94
Q

Where do the plaques form in asbestosis?

A

anterior and posterolateral parietal pleura;

DOMES OF THE DIAPHRAGM

95
Q

what type of pleural effusion happens in asbestosis?

A

serous

96
Q

T/F: pleural fibrosis is common later stages of asbestosis

A

false

97
Q

What is the catch phrase for sarcoidosis?

A

NONCASEATING GRANULOMAS in many tissues and organs

98
Q

What is the cause of sarcoid?

A

unknown

99
Q

Which race and which gender tend to get sarcoid?

A

females, blacks (10x more than whites)

100
Q

in which race is sarcoid rare?

A

asians

101
Q

Which cell mediates the immune response to sarcoid?

A

CD4+ T cells

102
Q

The genetic predisposition has familial and (blank) clustering

A

racial

103
Q

What are the possible environmental exposures that may cause sarcoid

A

Possibly a microbe (Rikettsia; Propionibacterium; mycobacteria)

104
Q

when looking at a lymph node with granulomas, how do you know if they are caseating or non-caseating?

A

the lesions that have red/darker in the middle are caseating; the singled colored/completely light lesions are non-caseating

105
Q

what type of biopsy can be used to Dx sarcoid?

A

transbronchial biopsy

106
Q

T/F: sarcoid causes honeycombing

A

true

107
Q

T/F: asbestosis causes honeycombing

A

true

108
Q

Explain how sarcoid leads to interstitial fibrosis

A

The granulomas, which are present in the interstitial space, result in fibrosis. So if granulomas are diffuse then possibly diffuse interstitial fibrosis can result.

109
Q

Which lymph nodes in the lung does sarcoid affect?

A

hilar and peribronchial;

110
Q

what will you see on CXR of sarcoid?

A

hilar and peribronchial LAD

111
Q

What are the pulmonary Sx that people have with sarcoid?

A

SOB
cough
chest pain
hemoptysis

112
Q

What are the two lab values that help indicate sarcoid?

A
  1. elevated ACE

2. Hypercalcemia

113
Q

why do pts with sarcoid have hypercalcemia?

A

granulomas release vit. D which increases Ca absorption

114
Q

in what percent of pts have a histologically affected spleen with sarcoid?

A

75%; 20% actually are enlarged

115
Q

after the spleen, what is the next organ to be affected in sarcoid?

A

liver

116
Q

What other disease may sarcoid mimmic?

A

Sjogrens; problems with salivary glands and tear production

117
Q

What is hypersensitivity pneumonitis?

A

Immune mediated, mostly interstitial lung disorders caused by abnormal sensitivity/reactivity to an inhaled organic antigen

118
Q

while asthma affects the bronchioles, hypersensitivity pneumonitis affects the….

A

alveoli

119
Q

What are the layman’s names for hypersensitivity pneumonitis?

A

farmer’s lung
pigeon breeder’s lung
Humidider/AC lung

120
Q

hypersensitivity pneumonitis is centered around which structure?

A

bronchiole, although it affects the alveoli

121
Q

The interstitial pneumonitis caused by hypersensitivity pneumonitis has an infiltrate of what three cell types?

A

lymphs
plasma cells
macrophages

122
Q

What type of granulomas does hypersensitivity pneumonitis show?

A

noncaseating!!

123
Q

T/F: hypersensitivity pneumonitis causes interstitial fibrosis

A

true

124
Q

T/F: hypersensitivity pneumonitis causes honeycombing

A

true

125
Q

T/F: hypersensitivity pneumonitis causes obliterative bronchiolitis

A

true; late though

126
Q

What is the difference between pneumonia and pneumonitis?

A 
Pneumonia= inflammation within alveoli
Pneumonitis = inflammation in the interstitium
127
Q

Describe the acute presentation of hypersensitivity pneumonitis?

A

FEVER

dyspnea, cough, leukocytosi 4-6 hours after exposure

128
Q

What are the Sx of chronic hypersensitivity pneumonitis?

A

respiratory failure
dyspnea
cyanoiss

129
Q

What part of the the exam is most important when thinking about hypersensitivity pneumonitis?

A

the history; symptoms get worse after cleaning the bird cage, or some such shit

130
Q

T/F: removal of the stimulus can prevent hypersensitivity pneumonitis from becoming a fibrotic disease

A

true

131
Q

what is the key feature in pulmonary eosinophilia?

A

RAPID ONSET FEVER

dsypnea, hypoxemia, resp failure

132
Q

what do you find in bronchio-alveolar lavage fluid in pulmonary eosinophilia?

A

25% eos

133
Q

Simple pulm. eosinophilia has (transient/perm.) lesions

A

transient

134
Q

T/F: simple pulm. eosinophilia is benign in its course

A

true

135
Q

besides in the BAL fluid, where else do you find eos in pulm. eosinophilia?

A

peripheral blood

136
Q

what are the lesions like in chronic eosinophilic pneumonia?

A

focal areas of consolidation with lymphs and eos

137
Q

What are the systemic symptoms of pulm. eosinophilia?

A

fever, night sweats, dsypnea

138
Q

T/F: pulmonary eosinophilia responds well to steroids

A

true

139
Q

Secondary eosinophilia can be caused by…

A

Usually due to infection, drug rxn, asthma, vasculitis, aspergillosis

140
Q

What is Pulmonary Alveolar Proteinosis?

A

Accumulation of acellular surfactant in the intra-alveolar and bronchiolar spaces

141
Q

Pulmonary Alveolar Proteinosis is identical to what other dz?

A

silicosis

142
Q

What are the three types of Pulmonary Alveolar Proteinosis?

A

Acquired
congenital
secondary

143
Q

T/F: Pulmonary Alveolar Proteinosis is fatal

A

true; 3-6 mo w/o transplant

144
Q

What are the causes of secondary Pulmonary Alveolar Proteinosis?

A

malignancies, immunodef, or SILICOSIS

145
Q

90% of all cases of Pulmonary Alveolar Proteinosis are what type?

A

acquired

146
Q

What is the MOA of the pathology of Pulmonary Alveolar Proteinosis?

A

Ab to GM-CSF; it is an autoimmune disorder

147
Q

WHat is the Tx for Pulmonary Alveolar Proteinosis?

A

GM-CSF supplementation: works in 50% of the cases

2. Whole lung lavage is standard of care.

Block 9 Week 3 (8 decks)

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