PATH: Obstructive and Restrictive Lung Disease Flashcards Preview

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Flashcards in PATH: Obstructive and Restrictive Lung Disease Deck (102):
1

List the obstructive lung diseases.

1) Emphysema
2) Chronic bronchitis
3) Bronchiectasis
4) Asthma

2

List the restrictive lung diseases.

1) Pneumoconiosis (Coal Worker's, Silicosis, Asbestosis)
2) Sarcoidosis
3) Pneumonias (various types)

3

What are the two divisions of obstructive lung disease?

Acute and Chronic

4

True or false: most COPD patients are smokers.

TRUE: 80%

5

True or false: eventually, most smokers develop COPD.

FALSE: only 15% of smokers get COPD

6

When does symptomatic COPD typically occur?

Middle age (but is dose dependent, so people who smoke more develop COPD earlier)

7

True or false: men are more likely to have COPD than women.

FALSE: prevalence is approximately equal between sexes

8

What is emphysema?

abnormal permanent enlargement of airspaces due to destruction of the walls (septa) between them—most commonly due to smoking

9

Who is at an increased risk for development of COPD (accounting for around 40,000 cases in 2 million Americans)?

Caucasian Americans with alpha-1-antitrypsin deficiency

10

Emphysema can be regarded as a disease of destructive ________, inadequate ______ control, and insufficient _______ _______.

Emphysema can be regarded as a disease of destructive inflammation, inadequate anti-inflammatory control and insufficient wound repair.

11

What cytokines are present in the ongoing inflammation of emphysema?

IL-8
TNF
Leukotriene B4

12

Without alpha-1-antitrypsin, what happens to alveolar wall?

Without this antiprotease, the alveolar wall becomes destroyed (even in the absence of smoking but emphysema is accelerated in smokers with the deficiency)

13

List genetic polymorphisms that can lead to inadequate repair of elastin and contribute to the development of emphysema.

High levels of:
MMP-9
MMP-12
Poor repair response to:
TGF-beta

14

At what point in a breath is a emphysema patient most likely to have collapsed airways? Why?

At expiration, the loss of elastic tissue reduces radial traction of the small airways and leads to collapse

15

How does emphysema lead to pulmonary hypertension?

loss of alveolar septal capillaries reduces pulmonary vascular capacitance--making the right heart pump the same amount of blood through a smaller network of vessels ( backing up pressure into the right right)

16

Emphysema can eventually lead to what disease (progresses from pulmonary HTN)?

Right heart failure (cor pulmonale)

17

Emphysema due to what is typically centriacinar?

smoking

Emphysema due to alpha-1-antitrypsin is typically panacinar

18

Emphysema due to smoking is typically centered around what structure?

respiratory bronchioles (spares alveoli)

19

Emphysema due to smoking is more severe in what lobes?

upper (especially apical segments)

20

Emphysema due to alpha-1-antitrypsin deficiency is more severe in what lobes?

lower

21

What type of emphysema is typical in young adult male smokers with spontaneous pneumothorax?

distal acinar and associated with massively enlarged subpleural airspaces (bullae) in upper lobes

22

What two words describe the microscopic pathology of emphysema?

enlarged airspaces

23

What is the most typical symptom of pure emphysema?

insidious onset of progressive dyspnea

24

What is the FEV1/FVC ratio for emphysema?

less than 0.7

25

What is chronic bronchitis?

productive cough for at least 3 months in 2 consecutive years in the absence of a specific diagnosis—mostly due to smoking

26

What is the pathogenesis of chronic bronchitis?

Toxins→ inflammation and hypersecretion of mucous/ hypertrophy of mucous secreting glands→ Obstruction

27

The inflammation associated with chronic bronchitis involves infiltration of what cell types?

CD8 Lymphocytes
Macrophages
Neutrophils

28

What leads to the mucous hypersecretion associated with chronic bronchitis?

T cell cytokines like IL-13 and increased transcription of the MUC5AC gene (MUCking up airways)

leads to hypertrophy of submucosal mucous-secreting glands and bronchial goblet cell hyperplasia

29

Describe the gross pathology of chronic bronchitis.

Bronchial mucosal hyperemia and edema with luminal mucinous or mucopurulent exudate

30

What will you see under the microscope of lung tissue with acute bronchitis?

-enlargement of submucosal glands
-lymphocyte infiltration
-goblet cell metaplasia in bronchioles
-luminal mucous plugs in bronchioles
-fibrosis in bronchioles

31

What is the only sign/symptom of chronic bronchitis?

productive cough

32

How do you diagnose chronic bronchitis?

H&P

33

How do you treat chronic bronchitis?

-Quit Smoking
-Short-acting, Inhaled Bronchodilators:
-Beta-2 agonist
-Anti-cholinergics
-Long-acting, Inhaled anti-cholinergics
-Inhaled corticocosteroidds

34

What is Bronchiectasis?

permanent dilation of bronchi due to destruction of muscle and elastic tissue by chronic or recurrent necrotizing infections

35

What three diseases can lead to the bronchial obstruction and chronic persistent infection that causes bronchiectasis?

1) Cystic Fibrosis
2) Immunodeficiency States
3) Kartagener syndrome

36

What is Kartagener syndrome?

rare AR disease causing ciliary impairment

37

What can lead to localized bronchiectasis?

bronchial tumor or necrotizing pneumonia

38

Describe the gross pathology of bronchiectasis.

Dilated bronchi (especially when close to visceral pleural surface); more pronounced in lower lobes

39

Describe the microscopic pathology of bronchiectasis.

scarred, dilated bronchi with intense acute and chronic inflammation

40

What type of sputum is coughed up with bronchiectasis?

copious purulent sputum

41

How do you diagnose bronchiectasis?

CT scan

42

How do you treat bronchiectasis?

long-term antibiotics

43

What is the definition of asthma?

chronic episodic obstructive airway disease due to reversible bronchoconstriction resulting from hyper-reactivity to various stimuli

44

What type of inflammation occurs in asthma?

Th2 type

45

What is involved with the early acute phase of Th2 type inflammation?

IgE
Mast Cells
Histamine
Leukotriene B4
Vagus nerve

46

What is involved with the late acute phase of Th2 type inflammation (4-8 hours after attack and lasting 24 hours max)?

Eosinophils
Neutrophils
Lymphocytes
IL-1, IL-6
TNF
Leukotrienes C4, D4, E4
PGD2
PAF

47

Describe the gross pathology of asthma.

Hyperinflated lungs +/- foci of atelectasis
Bronchial mucous plugs due to hypersecretion

48

Describe the microscopic pathology of asthma.

Bronchial luminal mucous and lots of neutrophils, the 3 C's, eosinophils, and submucosal edema (with mixed inflammatory infiltrate)

49

What are the 3 C's?

1) Curschmann's spirals
2) Charcot-Leyden crystals
3) Creola bodies

50

What are Curschmann's spirals?

small whorled mucous strands twisted in a common direction with a dense refractile coiled or braided core.

51

What are Charcot-Leyden crystals?

tiny crystals which are bipyramidal on longitudinal section and hexagonal on cross-section

52

What are Creola Bodies?

fragments of degenerated, sloughed respiratory epithelium

53

Describe the 6 changes that occur with airway remodeling.

1) Basement membrane thickening (2.5X larger)
2) Submucosal glandular hypertrophy/hyperplasia
3) Muscular wall hypertrophy and hyperplasia
4) Goblet cell metaplasia (and presence in bronchioles)
5) Bronchial epithelial hyperplasia
6) Submucosal thickening with fibrous tissue

54

How do you diagnose asthma?

History and Physical; spirometry

55

How do you treat asthma?

agonists; inhaled corticosteroids, antibody to IgE

56

What is pneumoconioses?

fibrosing restrictive lung diseases caused by inhalation of organic or inorganic particulates or chemical fumes

57

What is coal worker's pneumoconioses?

broad spectrum lung disease due to inhalation of coal dust ranging from asymptomatic non-fibrotic coal dust macules or fibrotic coal nodules to debilitating progressive massive pulmonary fibrosis

58

Black lung disease begins as what?

Anthracosis: Ingestion of carbon particles by alveolar macrophages

59

How does black lung disease progress from anthracosis?

inflammasome activation and IL-1 (due to non-carbon material in coal) can lead to fibrosis

60

What is the only way to definitively diagnose black lung?

lung exam by a pathologist

61

How can you treat black lung disease?

transplantation

62

What is silicosis?

slowly progressive nodular fibrosing lung disease due to inhalation of silicon dioxide in stone (especially quarts) or coal released by processes that powderize some of it (ex. sandblasting)

63

What is the pathogenesis of silicosis?

Ingestion of silica by alveolar macrophages→ release of TNF, IL-1, ROS, TGF-beta, fibronectin→ chronic inflammation

64

Describe the gross pathology of silicosis.

discrete palpable grey-tan nodules +/- anthracotic pigment

65

Describe the microscopic pathology of silicosis.

nodules of concentrically arranged hyalinized collagen

66

What interesting, yet non-specific feature of silicosis can be seen under the microscope?

weakly birefringent particles are visible under polarized light

67

What is asbestosis?

slowly progressive pulmonary interstitial fibrosis due to inhalation of asbestos fibers

68

Asbestosis is associated with what conditions?

associated with pleural plaques, lung cancers, and mesotheliomas

69

What is a strange feature of the epidemiology of asbestosis?

has a long latency so expected to peak in 2024

70

How does asbestosis occur?

amphibole type asbestos passes through tissue by gravity (have pointed ends that cannot be broken by macrophages and iron-coated ends that elicit a fibrosing tissue reaponse)

71

Describe the gross pathology of asbestosis.

interstitial pulmonary fibrosis (mostly at basal lower lobes), visceral pleural fibrosis, and well-circumscribed round/oval tan/white fibrous plaques on lower parietal pleura and domes of diaphragm

72

Describe the microscopic pathology of asbestosis.

interstitial fibrosis and ferruginous bodies of fibers

73

What is sarcoidosis?

non-caseating granulomatous multi-system inflammatory disease of unknown cause

74

Where is sarcoidosis most common (body-wise)?

most common in lung, lymph node, eye, and skin

75

What is the most likely cause of sarcoidosis?

Unknown—but most likely immunologic stimuli→ Th1 CD4 lymphocyte response

76

What is the role of IL-8 in sarcoidosis?

acts early as a chemo-attractant of neutrophils

77

Which interleukins promote the Th1 immune response of CD4 lymphocytes?

IL-12, IL-18, IL-27

78

Which interleukins mediate the transition to fibrosing inflammation? How?

IL-4 (it is a chemoattractant to fibroblasts)

79

Describe the gross pathology of sarcoidosis.

small tan non-necrotic granulomas in lymphangitic distribution along bronchovascular bundles and in bilateral hilar LN +/- interstitial fibrosis in lungs

80

Describe the microscopic pathology of sarcoidosis.

tight naked granulomas with giant cells (with asteroid bodies and Schaumann bodies) and epitheloid macrophages

81

What is an asteroid body?

stellate eosinophilic aggregates of cytoskeletal proteins within vacuoles

82

What is a Schaumann body?

laminated basophilic concretion of calcified proteins

83

25% of patients with sarcoidosis have what accompanying sign?

skin nodules (granulomas) or erythema nodosum

84

What is the differential diagnosis if you see reticulonodular pulmonary infiltrates and bilateral hilar lymphadenopahty on x-ray?

Sarcoidosis
Tb
Histoplasmosis

85

True of false: non-infectious interstitial lung disease is usually chronic.

TRUE

86

What is Usual Interstitial Pneumonia?

prototype chronic, slowly progressive, fibrosing inflammatory disease of the lungs involving predominantly the septa between air spaces

87

Describe the gross pathology of UIP.

fibrosis with large discrete scars worse in periphery and in lower lobes(lung is firmer and greyer)→ honey comb lung

88

Describe the microscopic pathology of UIP.

non-uniform patchy interstitial inflammation repair response and fibrosis with temporal heterogeneity

89

What is the hallmark of UIP?

fibroblast foci of immature fibrosis bulging into alveoli

90

What is temporal heterogeneity?

simulatneous presence of early, intermediate and late fibrosing lesions

91

What is the radiology diagnosis of UIP?

asymmetric bilateral irregular reticular(nodular) obacities at the bases and periphery with +/- a little ground-glass, traction bronchiectasis, honeycomb change

92

What is COP?

cryptogenic organizing pneumonia (bronchiolitis obliterans--organizing pneumonia) is a rare subacute fibrosing lung disease

93

COP is usually caused by what?

necrotizing infection (not cryptogenic)

94

What is the pathological hallmark of COP?

Masson bodies= plugs of fibrosing granulation tissue in alveoli and ducts

95

What do you see in a radiograph of COP?

bilateral opacities less dense than acute bacterial pneumonia or tumor→ ground glass and possibly air bronchograms

96

How can you treat COP and NSIP?

STEROIDS!!

97

What is NSIP?

non-specific interstitial pneumonia (but it IS specific)

98

What are the two types of NSIP?

Cellular (more inflammatory than fibrotic)
Fibrotic (more fibrotic than inflammatory)

99

What can you see in a radiograph of NSIP?

bilateral ground glass opacities

100

When does radiation pneumonitis occur?

1-2 months after radiation

101

What is the microscopic pathology of radiation pneumonitis?

Type two pneumocyte hyperplasia
Blood vessel injury
Residual hemosiderin (later)
Interstitial lymphocytes (later)
Active fibroblasts (later)
Interstitial fibrosis (later)

102

What part of the lung is particularly vulnerable to radiation injury?

small blood vessels