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Respiratory > Pathology of Restrictive Lung Disease > Flashcards

Pathology of Restrictive Lung Disease Flashcards

1
Q

What is the interstitium of the lung?

A

Connective tissue space around the airways and vessels and the space between the basement membranes of the alveolar walls

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2
Q

What is the connective tissue space around the airways and vessels called?

A

Interstitium of the lung

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3
Q

How are alveolar epithelial (pneumocytes) and interstitial capillary endothelial cell basement membranes in normal alveolar walls?

A

In direct contact

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4
Q

What does restrictive lung disease show?

A

Reduced lung compliance (stiff lungs)

Low FEV1 and low FVC but same FEV1/FVC ratio

Reduced gas transfer

Ventilation/perfusion mismatch

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5
Q

What does the spirometry of restrictive lung disease look like?

A
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6
Q

What is the presentation of restrictive lung disease?

A

Abnormal chest X-ray

Dyspnoea (on exertion and rest))

Respiratory failure (type 1)

Heart failure

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7
Q

What is dyspnoea?

A

Difficult breathing

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8
Q

What is difficult breathing called?

A

Dyspnoea

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9
Q

What is the difference in chest X-rays between:

Emphysema
Normal
Restrictive lung disease

A

Emphysema can see all ribs

Normal can see 10 ribs

Restrictive lung disease can see less than 10

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10
Q

What is restrictive lung disease also known as?

A

Interstitial lung disease

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11
Q

What is the pathway leading to restrictive lung disease?

A

1) Lung injury
2) Leads to chronic response
3) One of usual interstitial pneumonitis (UIP), granulomatous response, or other pattens leads to
4) Fibrosis or end stage honeycomb lung

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12
Q

What does the acute response to lung injury lead to?

A

Diffuse alveolar damage

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13
Q

What is diffuse alveolar damage associated with?

A

Major trauma

Chemical injury/toxic inhalation

Circulatory shock

Drugs

Infection

Autoimmune disease

Radiation

Idiopathic

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14
Q

What does idiopathic mean?

A

No known cause

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15
Q

What is no known cause called?

A

Idiopathic

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16
Q

What does the extend of diffuse alveolar damage depend on?

A

How many days the injury lasts for

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17
Q

What does DADS stand up for?

A

Diffuse alveolar damage

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18
Q

What are the histological features of acute alveolar damage?

A

Protein rich oedema

Fibrin

Hyaline membranes

Denuded basement membranes

Epithelial proliferation

Fibroblast proliferation

Scarring

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19
Q

Why do granulomatous responses to chronic inflammation lead to fibrosis or end stage honeycomb lung?

A

Sarcoidosis

Hypersensitivity pneumonitis

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20
Q

What is sarcoidosis?

A

Multisystem granulomatous disorder of unknown aetiology

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21
Q

What is a multisystem granulomatous disorder of known aetiology?

A

Sarcoidosis

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22
Q

What is the histopathology of sarcoidosis?

A

Epitheloid and giant cell granulomas

Necrosis/caseation very unusual

Little lymphoid infiltrate

Variable associated fibrosis

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23
Q

What is a granulomas?

A

Structure formed during inflammation that is found in many diseases, being a collection of macrophages

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24
Q

What is a structure formed during inflammation that is composed of a collection of macrophages?

A

Granulomas

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25
Q

What is the purpose of a granulomas?

A

Wall of a substance that cannot be destroyed

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26
Q

What is used to wall of a substance that cannot be destroyed?

A

Granulomas

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27
Q

Who does sarcoidosis commonly affect?

A

Young adults

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28
Q

Does sarcoidosis affect more males or females?

A

Females

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29
Q

What is the prevalence of sarcoidosis?

A

3-4/100,000 in UK

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30
Q

Where is sarcoidosis often common?

A

Temperate climates

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31
Q

What organs are involved in sarcoidosis?

A

Lymph nodes

Lung

Spleen

Liver

Skin, eyes, skeletal muscle

Bone marrow

Salivary glands

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32
Q

What are the most common organs involved in sarcoidosis?

A

Lymph nodes (100% of cases)

Lung (90% of cases)

Spleen (75% of cases)

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33
Q

What are typical presentations of sarcoidosis?

A

Young adult (acute arthralgia, erythema nodosum, bilateral hilar lymphadenopathy)

Incidental abnormal chest X-ray (no symptoms)

Shortness of breath, cough, and abnormal X-ray

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34
Q

What is acute arthralgia?

A

Joint pain

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35
Q

What is joint pain called?

A

Acute arthralgia

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36
Q

What is erythema nodosum?

A

Swollen fat under the skin that causes red bumps

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37
Q

What is swollen fat under the skin that causes red bumps called?

A

Erthema nodosum

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38
Q

What is bilateral hilar lymphadenopathy?

A

Bilateral enlargement of the lymph nodes of pulmonary hila

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39
Q

What is bilateral enlargement of the lymph nodes of pulmonary hila called?

A

Bilateral hilar lymphadenopathy

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40
Q

How long does it take for most presentations of sarcoidosis to resolve?

A

2 years

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41
Q

What are the possible outcomes of sarcoidosis after 2 years?

A

Resolve

Persist

Progress

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42
Q

What does the diagnosis of sarcoidosis involve?

A

Clinical findings

Imaging findings

Serum calcium and angiotenis converting enzyme (ACE)

Biopsy

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43
Q

What does ACE stand for?

A

Angiotenis converting enzyme

44
Q

What is hypersensitive pneumonitis?

A

Lung becomes inflammed due to an allergic reaction

45
Q

What is it called when the lung becomes inflammed due to an allergic reaction?

A

Hypersensitive pneumonitis

46
Q

What are possible antigens for hypersensitive pneumonitis?

A

Thermophillic actinomycetes

Bird/animal proteins (faeces)

Fungi (aspergillus species)

Chemicals

47
Q

What are the acute presentations of hypersensitive pneumonitis?

A

Fever, dry cough, myalgia

Chills 4-9 hours after antigen exposure

Crackles, tachyopnoea, wheeze

Precipitating antibody

48
Q

What is myalgia?

A

Pain in a muscle or group of muscles

49
Q

What is pain in a muscle or group of muscles called?

50
Q

What is tachypnoea?

A

Abnormally rapid breathing

51
Q

What is abnormally rapid breathing called?

A

Tachypnoea

52
Q

What are chronic presentations of hypersensitive pneumonitis?

A

Insidious

Malaise

Shortness of breath

Cough

Low grade illness

Crackles and some wheeze

53
Q

What is insidious?

A

Comes on slowly and does not have obvious symptoms at first

54
Q

What is something that comes in slowly and does not have obvious symptoms at first called?

55
Q

What is malaise?

A

General feeling of illness or discomfort

56
Q

What is the general feeling of illness or discomfort called?

57
Q

What can hypersentive pneumonitis lead to?

A

Respiratory failure due to gas transfer bing low

58
Q

What is the immune reaction of hypersensitive pneumonitis mediated by?

A

Type III and IV hypersensitive reaction

59
Q

What is the histopathology of hypersensitive pneumonitis?

A

Soft centriacinar epitheloid granulomata

Foamy histocytes

Bronchiolitis obliterans

Upper zone disease

60
Q

What are foamy histocytes?

A

Histocytes that are laden with lipid

61
Q

What are histocytes that are laden with lipid called?

A

Foamy histocytes

62
Q

What is bronchiolitis obliterans?

A

Disease results in obstruction of the small airways due to inflammation

63
Q

What does UIP stand up for?

A

Usual interstitial pneumonitis

64
Q

What is usual interstitial pneumonitis?

A

Form of lung disease characterised by progressive scarring of both lungs

65
Q

What is a form of lung disease characterised by progressive scarring of both lungs?

A

Usual interstitial pneumonitis (UIP)

66
Q

What may usual interstitial pneumonitis be seen in?

A

Connective tissue diseases

Drug reaction

Post infection

Industrial exposure (asbestos)

67
Q

What are examples of connective tissue diseases that can cause usual interstitial pneumonitis (UIP)?

A

Scleroderma

Rheutamoid

68
Q

What are most interstitial pneumonitis?

A

Cryptogenic or idiopathic

69
Q

What does cryptogenic mean?

A

Disease of obscure or uncertain origin

70
Q

What is a disease of obscure or uncertain called?

A

Cryptogenic

71
Q

What is the histology of usual interstitial pneumonitis?

A

Patchy interstitial chronic inflammation

Type II pneumocyte hyperplasia

Smooth muscle and vascular proliferation

Proliferating fibroblastic foci

72
Q

What is a hyperplasia?

A

Enlargement of an organ or tissue caused by an increase in the reproductive rate of its cells, of an initial stage in the development of cancer

73
Q

What is the enlargement of an organ or tissue caused by an increase in the reproductive rate of its cells called?

A

Hyperplasia

74
Q

What does normal pulmonary gas exchange look like?

A

Bulk flow

Beyond terminal bronchiole is diffusion

Blood-air barrier

Haemoglobin affinity for oxygen means blood leaving the capillary bed is 98% saturated for FIO2 of only 0.21

CO2 is very soluble and rapidly equilbrates between blood and air

75
Q

What are the different kinds of bulk flow?

A

Laminar

Turbulent

76
Q

What does bulk flow depend on?

A

Pressure difference

77
Q

What occurs beyond the terminal bronchiole?

78
Q

Why is blood 98% saturated at only 0.21 FIO2?

A

High affinity between haemoglobin and oxygen

80
Q

What is the normal PaO2?

A

10.5-13.5kPa

81
Q

What is the normal PaCO2?

82
Q

What are the 2 kinds of respiratory failure?

A

Type 1 (PaO2 < 8kPa)

Type 2 (PaCO2 > 6.5kPa)

83
Q

What are the 4 abnormal states associated with hypoxaemia?

A

Alveolar hypoventilation

Shunt

Ventilation/perfusion imbalance

Diffustion impairment

84
Q

What does hypoventilation do to partial pressure values?

A

Increases PACO2 so increases PaCO2

Increase in PACO2 decreases PAO2

Causes PaO2 to fall

85
Q

What is a fall in PaO2 due to alveolar hypoventilation fixed by?

A

Increasing FIO2

86
Q

What is shunt?

A

Blood passes from right to left side of the heart without contacting ventilated alveoli

87
Q

How large is a shunt normally?

88
Q

What diseases cause a shunt?

A

AV malformation

Congenital heart disease

Pulmonary disease

89
Q

How does a large shunt respond to increasing FIO2?

A

Poorly as blood leaving normal lung is already 98% saturated

90
Q

What is the size of a normal breath?

91
Q

What is the size of cardiac output?

92
Q

What is the normal V/Q?

93
Q

What is the most common cause of hypoxaemia?

94
Q

What does a low V/Q in only some alveoli arise due to?

A

Local alveolar hypoventilation due to some focal disease

95
Q

How do you treat a low V/Q?

A

Increase FIO2

96
Q

What does gas flow through a membrane depend on?

A

Thickness and surface area of the membrane and the gas pressure across it

97
Q

How does the speed of CO2 diffusion compare to oxygen?

A

20 times faster due to greater solubility

98
Q

What do diseases imparing gas diffusion not change?

A

CO2 levels

99
Q

What does diffusion impairment mean?

A

It takes longer for blood and alveolar air to equilbrate, particularly for oxygen

100
Q

How long does equilbrium of gas exchange normally take?

101
Q

What is the capillary transit time normally?

102
Q

What may occur when disease causes equilbrium to be closer to 0.75s?

A

PaO2 is maintained at rest but serious fall may occur during exercise

103
Q

How can hypoxaemia due to diffusion impairment be corrected?

A

Increasing FIO2

104
Q

What is hypoxaemia?

A

Abnormally low concentration of oxygen in the blood

105
Q

What is an abnormally low concentration of oxygen in the blood called?

A

Hypoxaemia

Respiratory (37 decks)

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