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All the things > 9.5 - 9.9: Restrictive Lung Diseases and rest of Respiratory > Flashcards

9.5 - 9.9: Restrictive Lung Diseases and rest of Respiratory Flashcards

1
Q

What happens to the TLC with restrictive lung diseases? FVC? FEV1?

A

All decrease, especially FVC

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2
Q

What happens to the FEV1:FVC ratio with restrictive diseases? Why?

A

Increased, since FVC falls more than FEV1

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3
Q

Why is it that the FEV1 does not fall very much with restrictive diseases?

A

Increased elastic recoil of the lungs causes an increase in the flow of air out of the lungs

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4
Q

Why do interstitial diseases lead to restrictive lung pathologies?

A

Fibrosis of the alveoli impede the opening of the alveolar sacs, as well as the gas exchange

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5
Q

What is idiopathic pulmonary fibrosis?

A

Fibrosis of the lung interstitium

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6
Q

What is the (known) etiology of idiopathic pulmonary fibrosis?

A

TGF-beta increased, causing cyclical lung injury and repair

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7
Q

What is the role of TGF-beta normally?

A

Encourages repair and growth

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8
Q

What are the two drugs that commonly cause lung injury?

A
  • Bleomycin

- Amiodarone

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9
Q

What are the s/sx of idiopathic pulmonary fibrosis?

A
  • Progressive dyspnea and cough

- Fibrosis on lung CT

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10
Q

What is the treatment for idiopathic pulmonary fibrosis?

A

Lung transplant–NOT steroids

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11
Q

Where in the lung does idiopathic pulmonary fibrosis occur?

A

Subpleural

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12
Q

What is pneumoconioses?

A

Interstitial fibrosis due to occupational exposure of small particles

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13
Q

What is the etiology of pneumoconiosis?

A

Small particles slip past mucosal defense and hit the alveoli, where macrophages freak out and induce fibrosis in the lung.

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14
Q

What is coal worker’s pneumoconioses? What happens to the lungs with this?

A

Carbon dust gets into the alveoli, causing massive fibrotic changes and shrunken lung

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15
Q

What is silicosis? Where in the lung does it occur, and how does it predispose to infx?

A

Silica sand pneumoconiosis usually occurring in the upper lobe of the lung, and impairs phagolysosome production in macrophages

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16
Q

What is berylliosis? What lung changes does this cause?

A
  • Be pneumoconiosis

- Noncaseating granulomas in the hilar lymph nodes

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17
Q

What is mesothelioma?

A

Asbestos pneumoconiosis

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18
Q

What is anthracosis?

A

Benign build up of carbon in macrophages d/t air pollution

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19
Q

What is the only pneumoconiosis that increases the risk for TB? Why?

A
  • Silicosis

- D/t impairment of the phagolysosome formation

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20
Q

Berylliosis is very similar to what other pathological condition? Why?

A
  • Sarcoidosis

- Noncaseating granuloma formation in the lungs and systemically

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21
Q

Which of the pneumoconioses poses an increased risk for the development of cancer?

A
  • Berylliosis

- Asbestos

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22
Q

Who is usually exposed to silica sand?

A

Sand blasters in construction

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23
Q

Who is usually exposed to beryllium?

A

Aerospace workers

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24
Q

Who is classically exposed to asbestos? What does it cause?

A
  • Shipyard workers or construction workers
  • Fibrosis of the lung/pleura
  • Cancer of the lung/pleura
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25
Q

Which is more common with asbestos exposure: mesothelioma, or lung cancer

A

Lung cancer

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26
Q

What are asbestos bodies?

A

Long rod-like Fe deposits, causing ferruginous bodies

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27
Q

What is sarcoidosis?

A

Systemic disease characterized by noncaseating granulomas in multiple organs

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28
Q

In whom is sarcoidosis usually seen?

A

African american females

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29
Q

What is the etiology of sarcoidosis?

A

Unknown, but likely due to CD4+ helper T cell response to an unknown antigen

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30
Q

What is the defining cell of granulomas?

A

Epithelioid histiocyte

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31
Q

What does non-caseating mean in the context of granulomas?

A

All of the cells in the granuloma are alive

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32
Q

What is the hallmark histological finding of sarcoidosis?

A

Asteroid body

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33
Q

Which parts of the lung are most commonly affected by sarcoidosis?

A

hilar lymph nodes

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34
Q

What type of lung disease does sarcoidosis eventually lead to?

A

Restrictive lung disease

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35
Q

Sarcoidosis of the eye causes what?

A

Uveitis

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36
Q

Sarcoidosis of the skin causes what?

A

Cutaneous nodules

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37
Q

Sarcoidosis of the salivary glands causes what?

A

Sjogren’s - like syndrome

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38
Q

What are the clinical features of sarcoidosis? (ssx, labs x2)

A
  • SOB/cough
  • Elevated ACE
  • Hypercalcemia
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39
Q

What is the treatment for sarcoidosis? Prognosis?

A

Steroids, but usually self limiting

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40
Q

Why is there hypercalcemia with sarcoidosis?

A

granulomas have alpha-1-hydroxylase activity, and can activate Vit D

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41
Q

What is hypersensitivity pneumonitis?

A

Granulomatous reaction to inhaled organic antigens

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42
Q

What are the s/sx of hypersensitivity pneumonitis?

A

Fever, cough, SOB

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43
Q

Chronic exposure to the antigen in hypersensitivity pneumonitis leads to what?

A

Interstitial fibrosis

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44
Q

—What are the cells that are classically found in the granulomas of hypersensitivity pneumonitis—

A

—Eosinophils—

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45
Q

What is the range of normal pulmonary BP? What defines pHTN?

A
  • Normal = 10 mmHg

- pHTN = more than 25 mmHg

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46
Q

What are the three major vessel changes that occur with pHTN?

A
  • Atherosclerosis of pulmonary trunk
  • Smooth muscle hypertrophy of pulmonary arteries
  • Intimal fibrosis
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47
Q

What are the hallmark lesions that are associated with chronic pHTN? What are these composed of?

A
  • Plexiform lesions

- Tufts of capillaries nestled together

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48
Q

What is the classic symptom of pHTN?

A

DOE

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49
Q

What is the heart sequelae of untreated pHTN?

A

RVH

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50
Q

Which gender is more classically affected with pHTN? What age (generally)?

A

Young Women

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51
Q

What is the genetic cause of the familial form of pHTN? What does this cause?

A

BMPR2, inactivating mutation, leading to proliferation of vascular smooth muscle

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52
Q

How does chronic hypoxemia (e.g. COPD, interstitial lung disease) lead to RVH and pHTN?

A

Increased capillary resistance

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53
Q

How do recurrent PEs cause pHTN?

A

Increasing pressure via blockage, or remodeling of the pulmonary artery

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54
Q

What is the histological change that occurs with ARDS? What happens to gas exchange?

A
  • Hyaline membrane formation d/t leaky capillaries and protein deposition
  • Leads to reduced gas exchange
55
Q

Why does diffuse collapse of the lung occur with ARDS?

A

Hyaline membranes stick to one another, causing alveoli to collapse

56
Q

What are the CXR findings of a patient with ARDS?

A

“White out” of the lung

57
Q

What are the s/sx of ARDS?

A

Hypoxemia and cyanosis with respiratory distress

58
Q

What are the cells that are damaged in ARDS?

A

Type I and II pneumocytes

59
Q

What are the immune cells that induce damage in ARDS?

A

Activated PMNs

60
Q

Why is PEEP necessary with ARDS?

A

Hyaline membranes will stick to one another and increase surface tension, so keeping in pressure will prevent from sticking

61
Q

What causes the interstitial fibrosis with ARDS?

A

Damage of the type II Pneumocytes (stem cells) means lung lining cannot heal well

62
Q

What is the cause of NRDS?

A

Inadequate surfactant levels

63
Q

What are the cells in the lungs that produce surfactant? What else do these cells do?

A
  • Type II pneumocytes

- Also the stem cells of the lung

64
Q

What is the use (benefit) of surfactant?

A

Reduce surface tension so less energy is needed to open alveoli

65
Q

What are the s/sx of NRDS?

A

Respiratory distress after birth, leading to cyanosis

66
Q

What are the classic CXR findings of NRDS?

A

Granularity of the lung

67
Q

What are the three major causes of NRDS?

A
  • Prematurity
  • C-section delivery
  • Maternal DM
68
Q

When in gestation does surfactant production begin? When is it adequate?

A

28 weeks

34 weeks is sufficient

69
Q

What are the two components of surfactant, and how is the ratio of these two used to determine if a child is ready to be born?

A

Lecithin and Sphingomyelin

L:S ratio greater than 2

70
Q

What is the major lipid found in surfactant?

A

Phosphatidylcholine (= Lecithin)

71
Q

Why can C-section delivery cause NRDS?

A

No release of endogenous corticosteroids to increase surfactant production (normally happens with stress from vaginal delivery)

72
Q

How can maternal DM cause NRDS?

A

Maternal BG levels will increase insulin levels in the fetus, which inhibit surfactant production

73
Q

What are the two major complications of NRDS? Why?

A
  • PDA (hypoxemia keeps it open)

- Necrotizing enterocolitis (hypoxia of the gut)

74
Q

—-What is the harm of administering oxygen to NRDS pts? What are the two major pathologies that can occur if this happens?—–

A
  • Free radical formation
  • Blindness (retinopathy of prematurity) and bronchopulmonary dysplasia
75
Q

What is the average age on presentation for lung CA?

A

60 years

76
Q

What are the three key environmental risk factors for the development of lung CA?

A
  • Cigarette smoke
  • Radon
  • asbestos
77
Q

What is the major carcinogenic component found in cigarettes?

A

Polycyclic aromatic hydrocarbons

78
Q

What is Radon formed from?

A

Decay product of Uranium

79
Q

What is the classic CXR finding of lung cancer?

A

Solitary “coin” lesion

80
Q

What is the first step when a nodule is found on a CXR?

A

Compare to priors

81
Q

Under what age are coin lesion more likely to be benign?

A

35 ish

82
Q

What are the causes of benign coin lesions? (2)

A
  • Granulomas

- Bronchial hamartoma

83
Q

Granulomas in the lung, particularly in the midwest, are often caused by what infectious agent?

A

Histoplasmosis

84
Q

What is a hamartoma?

A

Dysplastic tissue that belongs in that area, but is disorganized

85
Q

What are the two components of bronchial hamartomas?

A

Lung tissue and cartilage

86
Q

What are the two major classes of lung cancer?

A

Small cell and non-small cell

87
Q

True or false: small lung cell CA is almost never treated with surgery

A

True

88
Q

Cancerous mass in the lung that produces mucus and is glandular is what type of cancer?

A

Adenocarcinoma

89
Q

What are the key histological findings of squamous cell carcinoma?

A

Keratin pearls and intercellular bridges

90
Q

What are the top three cancers in the US by incidence?

A
  1. Breast/prostate
  2. Lung
  3. Colorectal
91
Q

What are the top three cancers in the US by mortality?

A
  1. Lung
  2. Prostate/breast
  3. Colorectal
92
Q

What are the histological characteristics of small cell lung carcinoma?

A

Poorly differentiated small cells that arise from neuroendocrine cells

93
Q

What are the histological characteristics of adenocarcinoma?

A

Glands or mucin production

94
Q

What are the histological characteristics of large cell carcinoma?

A

Poorly differentiated large cells (without other characteristic findings)

95
Q

What are the histological characteristics of bronchioloalveolar carcinoma? What cells do these arise from? Prognosis?

A
  • Columnar cells that grow along preexisting bronchioles and alveoli
  • Clara cells
  • Good prognosis
96
Q

What are the histological characteristics of carcinoid tumors (cell type, stain)?

A

Well differentiated neuroendocrine cells that are chromogranin positive

97
Q

What are the two most common cancers that metastasize to the lungs?

A

Breast

Colon

98
Q

Who usually gets small cell carcinoma?

A

Male smokers

99
Q

What is the most common form of cancer in male smokers?

A

Squamous cell carcinoma

100
Q

What is the most common form of lung cancer in female smokers?

A

Adenocarcinoma

101
Q

True or false: Bronchioloalveolar carcinoma is not related to smoking

A

True

102
Q

True or false: carcinoid tumors are related to smoking

A

False

103
Q

Central or peripherally located: Small cell carcinoma?

A

Central

104
Q

Central or peripherally located: squamous cell carcinoma?

A

Centrally

105
Q

Central or peripherally located: adenocarcinoma

A

Peripherally

106
Q

Central or peripherally located: large cell carcinoma

A

Central or peripheral

107
Q

Central or peripherally located: bronchioloalveolar carcinoma

A

peripherally

108
Q

Central or peripherally located: carcinoid tumors?

A

Either

109
Q

True or false: mets to the lung are more common than primary tumors

A

True

110
Q

True or false: carcinoid tumors are a low grade malignancy, but can cause carcinoid syndrome

A

True

111
Q

What are the typical CXR findings of bronchioloalveolar carcinoma? Prognosis?

A

Pneumonia-like consolidation

Excellent prognosis

112
Q

What endocrine signal may squamous cell carcinoma produce?

A

PTHrP

113
Q

What are the endocrine signals that small cell carcinomas usually produce?

A

ACTH or ADH

114
Q

What are the cells that give rise to small cell carcinomas?

A

Neuroendocrine cells (cells of Kulchitsky)

115
Q

What is Eaton-Lambert syndrome, and what lung cancer can cause this?

A

Antibodies against presynaptic Ca channels in the neuromuscular junction

116
Q

What is the mnemonic for remembering which lung cancers are related to smoking, and are centrally located?

A

If they start with the letter “S”, then they are related to “Smoking” and are “Sentrally” located

117
Q

What are the intercellular bridges seen in squamous cell carcinoma?

A

Desmosome connections

118
Q

What is the most common type of lung cancer in non-smokers? Females?

A

Adenocarcinoma

119
Q

Lung cancer that is chromogranin positive = ?

A

Carcinoid tumor

120
Q

Lung cancer that shows as a polyp-like mass in the bronchus = ?

A

Carcinoid tumor

121
Q

What is the TMN staging system of cancer?

A 
T = size and local extension
N = nodes
M = mets
122
Q

What organs usually receive mets from the lung?

A

Adrenal glands

123
Q

What lung cancer classically involves the pleura?

A

Adenocarcinoma

124
Q

How can the diaphragm or voice be affected with lung cancer?

A

Compression of the laryngeal or phrenic nerve

125
Q

—-What is a pancoast tumor?—-

A

—Lung cancer that compresses the sympathetic chain in the thorax, leading to Horner’s syndrome—-

126
Q

What type of cells line the pleura?

A

Mesothelial cells

127
Q

What is the most common cause of pneumothorax? In whom is this seen?

A

Rupture of an emphysematous bleb

Tall Young males

128
Q

What way does the trachea shift with a pneumothorax? Tension pneumothorax?

A

Toward in normal, away if tension

129
Q

What is the cause of tension pneumothoraces?

A

Penetrating chest wall injuries cause a hole whereby air can come in, but cannot leak out.

130
Q

What is the major complication that can occur with a tension pneumothorax?

A

Compression of the heart

131
Q

What are the usual presenting s/sx of mesothelioma?

A

Recurrent pleural effusions, dyspnea, and chest pain

132
Q

How does mesothelioma usually appear grossly? On imaging?

A
  • Completely surrounding the lung

- Plaques that surround the lung

133
Q

What type of cancer does chromogranin stain?

A

neuroendocrine tumors

134
Q

What is SVC syndrome?

A

Compression of the SVC by a pancoast tumor (classically), causing blue discoloration of the face, and edema

All the things (36 decks)

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