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Flashcards in Path x2 Deck (127):
1

What is bronchiectasis?

Permanent dilation of the bronchi and bronchioles, caused by destruction of muscle and elastin tissue

2

Is bronchiectasis reversible?

no

3

What are the two requisite conditions for bronchiectasis?

Obstruction and chronic, persistent infections

4

What is the histological change with bronchiectasis?

FIbrosis of the bronchioles, holding them open

5

What are the three common obstructive causes of bronchiectasis?

Tumor
FB
Concretions/secretions

6

What are the two congenital conditions that cause bronchiectasis?

CF
Kartagener's syndrome
Immunodeficiency

7

What type of pneumonia causes bronchiectasis? What organism?

Necrotizing
Staph Aureus, klebsiella, or TB

8

What chromosome is responsible in CF?

7

9

Why is there thick mucus with CF?

NaCl is pulled inward, opposite of sweat glands

10

What is the defect with Kartagener syndrome?

Structural defect in dynein of cilia

11

Is Kartagener syndrome AR or AD?

AR

12

What are the histological findings of the cilia with Kartagener syndrome?

Loss of the radial spokes

13

What are the ssx of bronchiectasis? (4)

Chronic cough
Foul smelling sputum
Hemoptysis
Dypsnea

14

What are the complications that can arise from bronchiectasis? (4)

Pulmonary HTN
Brain abscesses
Cor pulmonale
Amyloidosis

15

What is the major, basic issue with restrictive diseases?

Fibrosis of the lungs causes problems getting air in

16

What type of lung disease is kyphoscoliosis?

Restrictive

17

What are the two external causes of restrictive lung diseases?

Deformed chest wall
Pleural space filled with stuff

18

What are the hallmarks of chronic, diffuse interstitial diseases? What is the consequence of this?

Reduced compliance d/t inflammation and fibrosis.

Dyspnea results

19

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

TLC reduced
FEV1 normal-ish
FEV1/FVC normal

20

What is the typical presentation of interstitial lung diseases? Lung sounds?

SOB/hypoxia
Inspiratory crackles

21

What are the x-ray findings of interstitial lung disease?

Diffuse, bilateral infiltrative lesions, or ground glass shadows

22

What are the complications of interstitial lung diseases?

pHTN

23

What is the end stage result of ILDs?

honeycomb lung

24

What is acute lung injury?

Capillary damage causing non-cardiogenic pulmonary edema

25

What is the clinical presentation of acute lung injury?

Abrupt onset of significant hypoxemia and pulmonary infiltrates

26

What is ARDS?

Acute respiratory distress syndrome causing diffuse alveolar capillary damage

27

What are this histological manifestations of ARDs and acute lung injury?

Diffuse alveolar damage

28

What is the severe sequelae of ARDS?

Multisystem organ failure

29

What is the most common cause of noncardiogenic pulmonary edema?

ARDS

30

What are the three direct injures (from outside) that cause ARDS?

Infection
Aspiration
Oxygen toxicity

31

What are the four major indirect (from inside) causes of ARDS?

Shock
Sepsis
Toxins
TRALI

32

What are the CXR findings of ARDS?

bilateral infiltrates on CXR

33

What happens to pulmonary capillary wedge pressure with ARDS?

Is less than 18 mmHg

34

What happens to PaO2/FiO2 with ALI?

Less than 300

35

What happens to PaO2/FiO2 with ARDS?

Less than 200

36

What is the pathogenesis of ARDS?

Uncontrolled activation of acute inflammatory system, leading to an increased vascular permeability and alveolar thickening

37

What happens to diffusion capacity with ARDS?

Decreased

38

What happens to the surfactant with ARDS?

Widespread abnormalities

39

What causes the inflammation in endothelial cells with ARDS?

Complement and TNF-alpha

40

What causes the destruction of the alveolar capillaries with ARDS?

Oxygen radicals
Proteases
Prostaglandins

41

What is the primary effect of IL-8?

Potent PMN chemoattractant

42

What is the cause of IRDS?

Deficiency in pulmonary surfactant causes an increase in vascular permeability and alveolar flooding

43

What is the role of NO with ARDS?

Dilation of the pulmonary vasculature decreases PA pressure and resistance

44

What is the mortality rate with ARDS?

40%

45

What are the gross characteristics of the lungs with ARDS?

Heavy, thick, red, firm

46

What are the histological characteristics of ARDS? (2)

Interstitial and alveolar edema
*Hyaline membranes*

47

Transudate or exudate with ARDS?

Exudate

48

What are the three phases of ARDS, and when do they occur?

-Acute exudative: 0-7 days
-Proliferative phase 1-3 weeks
-fibrotic/healing 3-4 weeks

49

What happens in the fibrotic stage of ARDS? (what two cell types are activated)

Fibroblastic proliferation and type II pneumocyte hyperplasia

50

What is the general progression of ARDS?

1. injury = edema
2. Alveoli collapse, type II pneumocytes increase
3. Fibrosis

51

Do patients with ARDS always have fibrosis if they recover?

no

52

What is TRALI?

Anti-HLA or HNA antibodies cause lung collapse

53

What are the four major categories of ILD?

Fibrosing
Granulomatous
Eosinophilic
Smoking related

54

What are the three major fibrosing ILDs?

-Usual interstitial pneumonia (UIP)
-Non-specific interstitial pneumonia (NSIP)
-Cryptogenic organizing pneumonia (COP)

55

What are the two major granulomatous ILDs?

-Sarcoidosis
-Hypersensitivity pneumonitis

56

What is pneumoconiosis?

Inorganic material breathed in

57

What are the two smoking related ILDs?

DIP
Respiratory bronchiolitis

58

What is the initial finding of diffuse interstitial disease?

Alveolitis with Leukocyte accumulation

59

What is the final stage of diffuse interstitial lung diseases?

Fibrotic lung (honeycomb lung)

60

What is the role of M1 macrophages? M2?

M1 = Inflammation
M2 = healing

61

What is the major cell type that is implicated with diffuse interstitial disease?

Macrophages

62

What is idiopathic pulmonary fibrosis?

Pulmonary disorder of unknown etiology characterized by diffuse interstitial fibrosis

63

What is the histological pattern of idiopathic pulmonary fibrosis?

Usual interstitial pneumonia (UIP)

64

What is the term for the interstitial fibrosis with idiopathic pulmonary fibrosis?

Cryptogenic fibrosing alveolitis

65

What is the clinical course of idiopathic pulmonary fibrosis?

slow, Insidious onset of SOB with non-productive cough

66

What are the complications of late IPF?

pHTN

67

How do you diagnose IPF?

Diagnosis of exclusion

68

What is the prognosis of IPF? What is the treatment?

3 year survival

Lung transplant (NOT steroids)

69

What is the current theory of IPF?

Repeated cycles of epithelial activation/injury by some agent causes fibrosis

70

What is the major cell type that is upregulated with IPF? Cytokines?

Th2
IL-4
IL-5
IL-13

71

What is the hallmark histological finding of IPF? What causes this?

Fibroblastic foci

Overwhelming healing

72

What is the main fibrosing component of IPF? What does this do? (3)

TGF-beta1

-Activates fibroblasts and myofibroblasts
-Reduced telomerase
-Inhibits caveolin

73

What is the effect of TGF-beta1 on fibroblast caveolin in IPF? What does this cause?

TGF inhibits it

Caveolin can no longer inhibit deposition of collagen

74

What are the early findings of IPF? (2)

Alveolitis with leukocyte infiltration
"Fibroblastic foci"

75

What is the hallmark of UIP?

Patchy, interstitial fibrosis

76

What is the "temporal heterogeneity" seen with IPF?

Over time, lungs become more collagenous and less cellular, but occurring at different rates in different places

77

What are the late findings of IPF?

Dense fibrosis and collapse of alveolar wall

"honeycomb lung"

78

What causes the honeycomb lung in the end stages of lung diseases?

Dense fibrosis and collapse of the alveolar walls, leading to restructuring of airspaces and obliteration of small airways

79

What is the consequence of IPF?

cor pulmonale

80

What is the only form of ILD does not respond to steroids, and requires a transplant?

UIP

81

True or false: aspiration can lead to honeycomb lung

True

82

What are the diseases that can lead to honeycomb lung?

DAD
IPF
Interstitial granulomatous disease

83

What are the collagen vascular disease that can cause restrictive lung disease?

RA
Scleroderma
SLE

84

What restrictive lung disease can be caused by scleroderma?

NSIP

85

What is the main determinant of the pathogenesis of pneumoconioses?

Solubility and size

86

What does pneumoconiosis lead to?

Acute Lung Injury

87

What happens to larger particles in pneumoconioses?

Resist dissolution, leading to fibrosis

88

What is the physicochemical reactivity of the particles in lungs?

Direct tissue damage from releasing free radicals and other chemical groups

89

What is the key endogenous factor that determines the prognosis with pneumoconioses?

Capacity of inhaled dusts to stimulate fibrosis

90

What are the three main mediators release by macrophages that cause damage in pneumoconioses?

Free radicals
Chemotactic factors
Fibrogenic cytokines

91

What are the initial changes with coal exposure? What about for long term?

Emphysema progressing to fibrosis

92

What is the key characteristic of asbestos?

Extremely fibrogenic

93

What is anthracosis?

Benign buildup of carbon in macrophages d/t pollution

94

What is simple coal workers' pneumoconiosis (CWP)?

1-2 mm Nodules with collagen fibrils, but minimal lung dysfunction.

95

What is complicated CWP? What are the sequelae of this?

Progressive/massive fibrosis that leads to compromised lung function

Cor pulmonale from fibrosis and pHTN

96

Is there an increased risk for cancer or TB with CWP?

No

97

What is the eventual consequence of CWP?

Pulmonary massive fibrosis (PMF)

98

What is the "black lung disease"?

Disabling respiratory insufficiency d/t massive pulmonary fibrosis

99

What is Caplan syndrome? What does this lead to?

Coexistence of rheumatoid arthritis with a pneumoconiosis. Leads to the development of distinctive nodular pulmonary edema

100

What is the most prevalent occupational disease in the world?

Silicosis

101

What is the progression of silicosis?

Slowly progressive, nodular fibrosis

102

What are the ssx of silicosis?

Progressive SOB and cyanosis

103

What is the pathogenic part of silicosis?

Quartz crystal

104

What major cytokine is released when macrophages are exposed to the quartz in silicosis?

TNF
TGF beta

105

Are patients with silicosis more susceptible to cancer and/or TB?

Yes, to both

106

What are the gross characteristics of silicosis?

Concentrically arranged collagenous nodules that begin as small lesions in the upper lungs, but grow

107

What are the histological findings of silicosis? What technique can be used to highlight this?

Hyalinized whorls of collagen with scant inflammation

Polarized light

108

What are the serpentines seen with asbestos? How about amphiboles? Which is more pathogenic and is the one associated with mesothelioma?

Serpentines = Flexible curved rods
*Amphiboles* = Straight rods

109

Asbestos was classically seen in whom?

Construction yard or shipyard workers

110

Which type of pneumoconiosis causes pleural plaques and this pleural effusions?

Asbestosis

111

Which is more of an issue with asbestosis: mesothelioma or lung cancer?

Lung cancer develops much sooner

112

What is the histological pattern seen with asbestos? What else is seen?

UIP

Fibrosis and asbestos bodies

113

True or false: asbestos increases the risk for cancer systemically

True

114

What are the characteristics of the asbestos fibers that break through the interstitium to cause diffuse interstitial pulmonary fibrosis?

Large

115

What are asbestos body?

Asbestos fibers absorbed by macrophages and coated by hemosiderin

116

What is the general morphology of asbestosis? (what structures are affected/how does it progress)

DIffuse pulmonary fibrosis that begins around respiratory bronchioles and progressives to involve alveoli (honeycomb lung)

117

Which generally causes upper lobe fibrosis, and which causes lower lobe fibrosis: asbestosis, silicosis

Silicosis = upper lobe
Asbestosis = lower lobg

118

What is the increase in lung cancer development with asbestos exposure? What about with smoking? What abous with mesotheliomas?

5x with exposure
55x with smoking
1000x with mesothelioma

119

What causes the 1000x increase in lung CA with mesothelioma?

ROS generation with asbestos fibers

120

What is the clinical course of asbestosis?

-DOE, later at rest.
-Heart and respiratory failure

121

What are the drugs that cause lung fibrosis? (2)

Bleomycin
Amiodarone

122

What is the use of bleomycin?

Hodgkin's lymphoma

123

What is the use of methotrexate?

RA

124

What are the drugs that cause hypersensitivity pneumonitis? (2)

Methotrexate
Nitrofurantoin

125

What are the two drugs that cause bronchospasm?

ASA
Beta blockers

126

What is acute radiation pneumonitis?

1-6 months post radiation causes an inflammatory response, and can cause pleural effusions

127

What is chronic radiation pneumonitis

Failure of acute radiation pneumonitis to resolve, leading to pulmonary fibrosis