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01. Respiratory System - HP > - Pathology of Restrictive Lung Disease > Flashcards

- Pathology of Restrictive Lung Disease Flashcards

1
Q

<p>What is the interstitium of the lung?</p>

A

<p>Connective tissue space around the airways and vessels and the space between the basement membranes of the alveolar walls</p>

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2
Q

<p>What is the connective tissue space around the airways and vessels called?</p>

A

<p>Interstitium of the lung</p>

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3
Q

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

A

<p>In direct contact</p>

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4
Q

<p>What does restrictive lung disease show?</p>

A

<p>Reduced lung compliance (stiff lungs)</p>

<p>Low FEV1and low FVC but same FEV1/FVC ratio</p>

<p>Reduced gas transfer</p>

<p>Ventilation/perfusion mismatch</p>

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5
Q

What does the spirometry of restrictive lung disease look like ?

A
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6
Q

<p>What is the presentation of restrictive lung disease?</p>

A

<p>Abnormal chest X-ray</p>

<p>Dyspnoea (on exertion and rest))</p>

<p>Respiratory failure (type 1)</p>

<p>Heart failure</p>

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7
Q

<p>What is dyspnoea?</p>

A

<p>Difficult breathing</p>

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8
Q

<p>What is difficult breathing called?</p>

A

<p>Dyspnoea</p>

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9
Q

<p>What is the difference in chest X-rays between:</p>

<p>Emphysema<br></br>Normal<br></br>Restrictive lung disease</p>

A

<p>Emphysema can see all ribs</p>

<p>Normal can see 10 ribs</p>

<p>Restrictive lung disease can see less than 10</p>

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10
Q

<p>What is restrictive lung disease also known as?</p>

A

<p>Interstitial lung disease</p>

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11
Q

<p>What is the pathway leading to restrictive lung disease?</p>

A

<p>1) Lung injury</p>

<p>2) Leads to chronic response</p>

<p>3) One of usual interstitial pneumonitis (UIP), granulomatous response, or other pattens leads to</p>

<p>4) Fibrosis or end stage honeycomb lung</p>

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12
Q

<p>What does the acute response to lung injury lead to?</p>

A

<p>Diffuse alveolar damage</p>

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13
Q

<p>What is diffuse alveolar damage associated with?</p>

A

<p>Major trauma</p>

<p>Chemical injury/toxic inhalation</p>

<p>Circulatory shock</p>

<p>Drugs</p>

<p>Infection</p>

<p>Autoimmune disease</p>

<p>Radiation</p>

<p>Idiopathic</p>

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14
Q

<p>What does idiopathic mean?</p>

A

<p>No known cause</p>

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15
Q

<p>What is no known cause called?</p>

A

<p>Idiopathic</p>

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16
Q

<p>What does the extend of diffuse alveolar damage depend on?</p>

A

<p>How many days the injury lasts for</p>

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17
Q

<p>What does DADS stand up for?</p>

A

<p>Diffuse alveolar damage</p>

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18
Q

<p>What are the histological features of acute alveolar damage?</p>

A

<p>Protein rich oedema</p>

<p>Fibrin</p>

<p>Hyaline membranes</p>

<p>Denuded basement membranes</p>

<p>Epithelial proliferation</p>

<p>Fibroblast proliferation</p>

<p>Scarring</p>

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19
Q

<p>Why do granulomatous responses to chronic inflammation lead to fibrosis or end stage honeycomb lung?</p>

A

<p>Sarcoidosis</p>

<p>Hypersensitivity pneumonitis</p>

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20
Q

<p>What is sarcoidosis?</p>

A

<p>Multisystem granulomatous disorder of unknown aetiology</p>

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21
Q

<p>What is a multisystem granulomatous disorder of known aetiology?</p>

A

<p>Sarcoidosis</p>

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22
Q

<p>What is the histopathology of sarcoidosis?</p>

A

<p>Epitheloid and giant cell granulomas</p>

<p>Necrosis/caseation very unusual</p>

<p>Little lymphoid infiltrate</p>

<p>Variable associated fibrosis</p>

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23
Q

<p>What is a granulomas?</p>

A

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

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24
Q

<p>What is a structure formed during inflammation that is composed of a collection of macrophages?</p>

A

<p>Granulomas</p>

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25
Q

<p>What is the purpose of a granulomas?</p>

A

<p>Wall of a substance that cannot be destroyed</p>

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26
Q

<p>What is used to wall of a substance that cannot be destroyed?</p>

A

<p>Granulomas</p>

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27
Q

<p>Who does sarcoidosis commonly affect?</p>

A

<p>Young adults</p>

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28
Q

<p>Does sarcoidosis affect more males or females?</p>

A

<p>Females</p>

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29
Q

<p>What is the prevalence of sarcoidosis?</p>

A

<p>3-4/100,000 in UK</p>

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30
Q

<p>Where is sarcoidosis often common?</p>

A

<p>Temperate climates</p>

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31
Q

<p>What organs are involved in sarcoidosis?</p>

A

<p>Lymph nodes</p>

<p>Lung</p>

<p>Spleen</p>

<p>Liver</p>

<p>Skin, eyes, skeletal muscle</p>

<p>Bone marrow</p>

<p>Salivary glands</p>

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32
Q

<p>What are the most common organs involved in sarcoidosis?</p>

A

<p>Lymph nodes (100% of cases)</p>

<p>Lung (90% of cases)</p>

<p>Spleen (75% of cases)</p>

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33
Q

<p>What are typical presentations of sarcoidosis?</p>

A

<p>Young adult (acute arthralgia, erythema nodosum, bilateral hilar lymphadenopathy)</p>

<p>Incidental abnormal chest X-ray (no symptoms)</p>

<p>Shortness of breath, cough, and abnormal X-ray</p>

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34
Q

<p>What is acute arthralgia?</p>

A

<p>Joint pain</p>

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35
Q

<p>What is joint pain called?</p>

A

<p>Acute arthralgia</p>

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36
Q

<p>What is erythema nodosum?</p>

A

<p>Swollen fat under the skin that causes red bumps</p>

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37
Q

<p>What is swollen fat under the skin that causes red bumps called?</p>

A

<p>Erthema nodosum</p>

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38
Q

<p>What is bilateral hilar lymphadenopathy?</p>

A

<p>Bilateral enlargement of the lymph nodes of pulmonary hila</p>

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39
Q

<p>What is bilateral enlargement of the lymph nodes of pulmonary hila called?</p>

A

<p>Bilateral hilar lymphadenopathy</p>

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40
Q

<p>How long does it take for most presentations of sarcoidosis to resolve?</p>

A

<p>2 years</p>

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41
Q

<p>What are the possible outcomes of sarcoidosis after 2 years?</p>

A

<p>Resolve</p>

<p>Persist</p>

<p>Progress</p>

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42
Q

<p>What does the diagnosis of sarcoidosis involve?</p>

A

<p>Clinical findings</p>

<p>Imaging findings</p>

<p>Serum calcium and angiotenis converting enzyme (ACE)</p>

<p>Biopsy</p>

43
Q

<p>What does ACE stand for?</p>

A

<p>Angiotenis converting enzyme</p>

44
Q

<p>What is hypersensitive pneumonitis?</p>

A

<p>Lung becomes inflammed due to an allergic reaction</p>

45
Q

<p>What is it called when the lung becomes inflammed due to an allergic reaction?</p>

A

<p>Hypersensitive pneumonitis</p>

46
Q

<p>What are possible antigens for hypersensitive pneumonitis?</p>

A

<p>Thermophillic actinomycetes</p>

<p>Bird/animal proteins (faeces)</p>

<p>Fungi (aspergillus species)</p>

<p>Chemicals</p>

47
Q

<p>What are the acute presentations of hypersensitive pneumonitis?</p>

A

<p>Fever, dry cough, myalgia</p>

<p>Chills 4-9 hours after antigen exposure</p>

<p>Crackles, tachyopnoea, wheeze</p>

<p>Precipitating antibody</p>

48
Q

<p>What is myalgia?</p>

A

<p>Pain in a muscle or group of muscles</p>

49
Q

<p>What is pain in a muscle or group of muscles called?</p>

A

<p>Myalgia</p>

50
Q

<p>What is tachypnoea?</p>

A

<p>Abnormally rapid breathing</p>

51
Q

<p>What is abnormally rapid breathing called?</p>

A

<p>Tachypnoea</p>

52
Q

<p>What are chronic presentations of hypersensitive pneumonitis?</p>

A

<p>Insidious</p>

<p>Malaise</p>

<p>Shortness of breath</p>

<p>Cough</p>

<p>Low grade illness</p>

<p>Crackles and some wheeze</p>

53
Q

<p>What is insidious?</p>

A

<p>Comes on slowly and does not have obvious symptoms at first</p>

54
Q

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

A

<p>Insidious</p>

55
Q

<p>What is malaise?</p>

A

<p>General feeling of illness or discomfort</p>

56
Q

<p>What is the general feeling of illness or discomfort called?</p>

A

<p>Malaise</p>

57
Q

<p>What can hypersentive pneumonitis lead to?</p>

A

<p>Respiratory failure due to gas transfer bing low</p>

58
Q

<p>What is the immune reaction of hypersensitive pneumonitis mediated by?</p>

A

<p>Type III and IV hypersensitive reaction</p>

59
Q

<p>What is the histopathology of hypersensitive pneumonitis?</p>

A

<p>Soft centriacinar epitheloid granulomata</p>

<p>Foamy histocytes</p>

<p>Bronchiolitis obliterans</p>

<p>Upper zone disease</p>

60
Q

<p>What are foamy histocytes?</p>

A

<p>Histocytes that are laden with lipid</p>

61
Q

<p>What are histocytes that are laden with lipid called?</p>

A

<p>Foamy histocytes</p>

62
Q

<p>What is bronchiolitis obliterans?</p>

A

<p>Disease results in obstruction of the small airways due to inflammation</p>

63
Q

<p>What does UIP stand up for?</p>

A

<p>Usual interstitial pneumonitis</p>

64
Q

<p>What is usual interstitial pneumonitis?</p>

A

<p>Form of lung disease characterised by progressive scarring of both lungs</p>

65
Q

<p>What is a form of lung disease characterised by progressive scarring of both lungs?</p>

A

<p>Usual interstitial pneumonitis (UIP)</p>

66
Q

<p>What may usual interstitial pneumonitis be seen in?</p>

A

<p>Connective tissue diseases</p>

<p>Drug reaction</p>

<p>Post infection</p>

<p>Industrial exposure (asbestos)</p>

67
Q

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

A

<p>Scleroderma</p>

<p>Rheutamoid</p>

68
Q

<p>What are most interstitial pneumonitis?</p>

A

<p>Cryptogenic or idiopathic</p>

69
Q

<p>What does cryptogenic mean?</p>

A

<p>Disease of obscure or uncertain origin</p>

70
Q

<p>What is a disease of obscure or uncertain called?</p>

A

<p>Cryptogenic</p>

71
Q

<p>What is the histology of usual interstitial pneumonitis?</p>

A

<p>Patchy interstitial chronic inflammation</p>

<p>Type II pneumocyte hyperplasia</p>

<p>Smooth muscle and vascular proliferation</p>

<p>Proliferating fibroblastic foci</p>

72
Q

<p>What is a hyperplasia?</p>

A

<p>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</p>

73
Q

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

A

<p>Hyperplasia</p>

74
Q

<p>What does normal pulmonary gas exchange look like?</p>

A

<p>Bulk flow</p>

<p>Beyond terminal bronchiole is diffusion</p>

<p>Blood-air barrier</p>

<p>Haemoglobin affinity for oxygen means blood leaving the capillary bed is 98% saturated for FIO2of only 0.21</p>

<p>CO2is very soluble and rapidly equilbrates between blood and air</p>

75
Q

<p>What are the different kinds of bulk flow?</p>

A

<p>Laminar</p>

<p>Turbulent</p>

76
Q

<p>What does bulk flow depend on?</p>

A

<p>Pressure difference</p>

77
Q

<p>What occurs beyond the terminal bronchiole?</p>

A

<p>Diffusion</p>

78
Q

<p>Why is blood 98% saturated at only 0.21 FIO2?</p>

A

<p>High affinity between haemoglobin and oxygen</p>

79
Q

<p>What is the normal PaO2?</p>

A

<p>10.5-13.5kPa</p>

80
Q

<p>What is the normal PaCO2?</p>

A

<p>4.8-6kPa</p>

81
Q

<p>What are the 2 kinds of respiratory failure?</p>

A

<p>Type 1 (PaO2< 8kPa)</p>

<p>Type 2 (PaCO2> 6.5kPa)</p>

82
Q

<p>What are the 4 abnormal states associated with hypoxaemia?</p>

A

<p>Alveolar hypoventilation</p>

<p>Shunt</p>

<p>Ventilation/perfusion imbalance</p>

<p>Diffustion impairment</p>

83
Q

<p>What does hypoventilation do to partial pressure values?</p>

A

<p>Increases PACO2so increases PaCO2</p>

<p>Increase in PACO2decreases PAO2</p>

<p>Causes PaO2to fall</p>

84
Q

<p>What is a fall in PaO2due to alveolar hypoventilation fixed by?</p>

A

<p>Increasing FIO2</p>

85
Q

<p>What is shunt?</p>

A

<p>Blood passes from right to left side of the heart without contacting ventilated alveoli</p>

86
Q

<p>How large is a shunt normally?</p>

A

<p>2-4%</p>

87
Q

<p>What diseases cause a shunt?</p>

A

<p>AV malformation</p>

<p>Congenital heart disease</p>

<p>Pulmonary disease</p>

88
Q

<p>How does a large shunt respond to increasing FIO2?</p>

A

<p>Poorly as blood leaving normal lung is already 98% saturated</p>

89
Q

<p>What is the size of a normal breath?</p>

A

<p>4L/min</p>

90
Q

<p>What is the size of cardiac output?</p>

A

<p>5L/min</p>

91
Q

<p>What is the normal V/Q?</p>

A

<p>0.8</p>

92
Q

<p>What is the most common cause of hypoxaemia?</p>

A

<p>Low V/Q</p>

93
Q

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

A

<p>Local alveolar hypoventilation due to some focal disease</p>

94
Q

<p>How do you treat a low V/Q?</p>

A

<p>Increase FIO2</p>

95
Q

<p>What does gas flow through a membrane depend on?</p>

A

<p>Thickness and surface area of the membrane and the gas pressure across it</p>

96
Q

<p>How does the speed of CO2diffusion compare to oxygen?</p>

A

<p>20 times faster due to greater solubility</p>

97
Q

<p>What do diseases imparing gas diffusion not change?</p>

A

<p>CO2levels</p>

98
Q

<p>What does diffusion impairment mean?</p>

A

<p>It takes longer for blood and alveolar air to equilbrate, particularly for oxygen</p>

99
Q

<p>How long does equilbrium of gas exchange normally take?</p>

A

<p>0.25s</p>

100
Q

<p>What is the capillary transit time normally?</p>

A

<p>0.75s</p>

101
Q

<p>What may occur when disease causes equilbrium to be closer to 0.75s?</p>

A

<p>PaO2is maintained at rest but serious fall may occur during exercise</p>

102
Q

<p>How can hypoxaemia due to diffusion impairment be corrected?</p>

A

<p>Increasing FIO2</p>

103
Q

<p>What is hypoxaemia?</p>

A

<p>Abnormally low concentration of oxygen in the blood</p>

104
Q

<p>What is an abnormally low concentration of oxygen in the blood called?</p>

A

<p>Hypoxaemia</p>

01. Respiratory System - HP (82 decks)

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