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Respiratory > Restrictive lung disease - Full summary > Flashcards

Restrictive lung disease - Full summary Flashcards

1
Q

What is meant by restrictive lung disease?

A

Any disease in which there is a reduced ability of the lungs to expand to take in air

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2
Q

What are the 2 main categories of restrictive lung disease?

A

Intrinsic
Extrinsic

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3
Q

What is meant by intrinsic restrictive lung disease?

A

Any restrictive lung disease caused by a disease of the lung itself, most commonly the alveoli

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4
Q

What is meant by extrinsic restrictive lung disease?

A

Any restrictive lung disease caused by a disease of the structures surrounding the lungs, which prevents expansion

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5
Q

How do intrinsic restrictive lung diseases affect O2 and CO2 exchange?

A

It affects the diffusion barrier and so can affect O2 intake, however, CO2 exchange will usually be unimpaired as exhalation is still normal, so CO2 is still blown off

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6
Q

What are some diseases that increase pressure on the thorax and so can cause extrinsic restrictive lung disease?

A

Obesity
Kyphoscoliosis
Ascites (Fluid in peritoneal cavity)
Diaphragmatic palsy

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7
Q

What are some ways in which diseases can cause extrinsic restrictive lung diseases?

A

Increased thoracic pressure
Reduction of thoracic muscle contraction
Pleural diseases affecting lung compliance

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8
Q

What are some diseases affecting thoracic muscle contraction that can lead to extrinsic restrictive lung disease?

A

Motor neurone disease
Myasthenia gravis

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9
Q

What are some diseases that can affect the pleura and thus cause extrinsic restrictive lung disease?

A

Diffuse pleural thickening
Mesothelioma
Large pleural effusions

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10
Q

What is the first line test performed in suspected restrictive lung disease?

A

Pulmonary function testing

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11
Q

What will be shown on pulmonary function testing (Spirometry) in restrictive lung disease?

A

A restrictive pattern:
Decreased FVC - Less air into lungs
Maintained FEV1 - Rate of contraction unaffected
Therefore FER > 80%

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12
Q

What is meant by DLCO?

A

Diffusion Capacity of the Lungs to CO

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13
Q

How is DLCO affected in those with restrictive lung disease?

A

It will be decreased below 80%

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14
Q

What are some conditions that can result in decreased DLCO?

A

Restrictive lung disease
Anaemia
Emphysema
Pulmonary oedema
Pulmonary embolism
Asthma

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15
Q

What PaO2 level shows type I respiratory failure?

A

PaO2 < 8Kpa

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16
Q

What pCO2 levels shows type II respiratory failure?

A

pCO2 > 6kPa

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17
Q

Which form of respiratory failure can be seen in restrictive lung disease?

A

Both type I or type II

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18
Q

What are some 2nd line tests that can be performed in restrictive lung disease?

A

Chest X-ray
CT scanning
Pleural ultrasound
Connective tissue screen
Vasculitis screen

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19
Q

What will be seen on blood testing in restrictive lung disease?

A

Polycythaemia from chronic hypoxia

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20
Q

What are some common symptoms of CO2 retention in restrictive lung disease?

A

Headaches
Confusion
Lethargy

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21
Q

What are some common clinical findings in restrictive lung disease?

A

Finger clubbing
Obesity
Kyphoscoliosis
Fibrotic crepitations
Signs of pleural effusion or ascites
Cyanosis

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22
Q

What are some common clinical signs of CO2 retention in restrictive lung disease?

A

Flushed skin
Bounding pulse
Rapid breathing
Premature heart beat
Muscle twitches
Flapping tremor

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23
Q

What are some lifestyle management techniques in restrictive lung disease?

A

Improved diet and exercise
Pulmonary rehabilitation
Weight loss

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24
Q

How is myasthenia graves pharmacologically treated in restrictive lung disease?

A

Pyridostigmine

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25
Q

What are some possible surgical and clinical procedures in restrictive lung disease?

A

Intercostal drainage in pleural effusion
Ascitic drainage in ascites
Corrective spinal surgery in scoliosis
Decortication in chronic empyema
Diaphragmatic plication in diaphragmatic palsy
Lung transplant in pulmonary fibrosis

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26
Q

What is the most common form of intrinsic restrictive lung disease?

A

Interstitial lung disease

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27
Q

What are the 4 main classes of interstitial lung disease?

A

ILD of known cause
Idiopathic interstitial pneumonia
Granulomatous ILD
Other forms of ILD

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28
Q

What are some examples of granulomatous (Inflammatory) interstitial lung diseases?

A

Sarcoidosis
Hypersensitivity pneumonitis

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29
Q

What is an example of a disease that falls within the “other” category of interstitial lung disease?

A

Lymphangioleiomyomatosis (LAM)

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30
Q

What are the 2 main classes of idiopathic pulmonary pneumonia?

A

Idiopathic pulmonary fibrosis
Idiopathic interstitial pneumonia

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31
Q

What are some examples of idiopathic interstitial pneumonias?

A

Desquamative interstitial pneumonia
Respiratory bronchiolitis ILD
Acute interstitial pneumonia
Cryptogenic organising pneumonia
Non-specific interstitial pneumonia
Lymphocytic interstitial pneumonia

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32
Q

What is meant by interstitial lung disease?

A

Any disease affecting the lung interstitial (e.g. alveoli and terminal bronchi)

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33
Q

What are some symptoms of interstitial lung disease?

A

Breathlessness
Dry cough

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34
Q

How can symptoms of interstitial lung disease present?

A

Acutely
Episodically
Chronically

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35
Q

What type of disease is sarcoidosis?

A

Sarcoidosis sis a granulomatous, type 4 hypersensitivity disease of unknown cause

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36
Q

Where are the most common locations of granuloma formation in sarcoidosis?

A

Lungs
Mediastinal lymph nodes
Joints
Liver
Skin
Eyes

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37
Q

What are some less common locations of granuloma formation in sarcoidosis?

A

Kidneys
Brain
Nerves
Heart

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38
Q

Describe the pathology of granulomas in sarcoidosis

A

Non-caseating (Non-Cheesy)

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39
Q

Are smokers at a higher or lower risk of sarcoidosis?

A

Higher

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40
Q

What are the 2 main types of sarcoidosis?

A

Acute
Chronic

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41
Q

How does acute sarcoidosis present?

A

Erythema nodosum
Lupus pernio
Bilateral hillar lymphadenopathy
Arthritis
Anterior chamber uveitis
Glaucoma
Parotitis
Fever

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42
Q

What is meant by erythema nodosum?

A

Raised, tender nodules on the shins

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43
Q

What is meant by lupus pernio?

A

Raised red lesions on the face

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44
Q

How does chronic sarcoidosis present?

A

Lung infiltrates - Alveolitis
Skin infiltrations
Peripheral lymphadenopathy
Hypercalcaemia

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45
Q

What is the cause of hypercalcaemia in sarcoidosis?

A

Vitamin C breakdown in granulomas causes an increase in Ca2+ levels

46
Q

What are some conditions that sarcoidosis can mimic?

A

Lymphoma
Tuberculosis
Carcinoma
Fungal infection

47
Q

What is the first line test in diagnosis of sarcoidosis?

A

Chest X-ray to check for bilateral hilarity lymphadenopathy

48
Q

What are some other tests that can be carried out in diagnosis of sarcoidosis?

A

CT scan of lungs - Nodular infiltrates
Tissue biopsy for granulomas
PFTs
Blood tests - High ACE levels
Raised Ca2+
Raised inflammatory markers

49
Q

How should acute sarcoidosis with no end organ damage be treated?

A

This is usually self limiting, so no treatment is usually given

50
Q

How should acute sarcoidosis with end organ damage be treated?

A

This is usually treated with oral steroids such as prednisolone

51
Q

How is chronic sarcoidosis usually treated?

A

Oral steroids (e.g. prednisolone)
Possible immunosuppression (e.g. methotrexate, azathioprine, anti-TNF therapy)

52
Q

What is the old name for hypersensitivity pneumonitis?

A

Extrinsic Allergic Alveolitis

53
Q

What is hypersensitivity pneumonitis?

A

A type III hypersensitivity reaction to an antigen deposited in the lungs, however, there can be some type IV granuloma formation

54
Q

What causes hypersensitivity pneumonitis?

A

Inhaled particulates or drugs

55
Q

What are some examples of inhaled particles that can cause hypersensitivity pneumonitis?

A

Thermophilic actinomycetes
Avian antigens

56
Q

What are thermophilic actinomycetes?

A

These are unique, high temperature aerobic bacteria

57
Q

What are some forms of hypersensitivity pneumonitis caused by thermophilic actinomycetes?

A

Farmer’s lung
Malt worker’s lung
Mushroom worker’s lung

58
Q

What form of hypersensitivity pneumonitis caused by inhalation of avian antigens?

A

Bird fancier’s lung

59
Q

Who is most at risk of bird fancier’s lung?

A

Those who own pet birds
Those with feather down duvets or pillows

60
Q

What are some drugs that can cause hypersensitivity pneumonitis?

A

Gold
Bleomycin
Sulphasalazine

61
Q

What are the 2 main classes of hypersensitivity pneumonitis?

A

Acute
Chronic

62
Q

What are some symptoms of acute hypersensitivity pneumonitis?

A

Cough
Breathlessness
Fever
Myalgia

63
Q

When do symptoms of acute hypersensitivity pneumonitis usually occur?

A

Symptoms often occur several hours after exposure

64
Q

What are some clinical signs of acute hypersensitivity pneumonitis?

A

Pyrexia
Crackles
No wheeze
Hypoxia

65
Q

What are some symptoms of chronic hypersensitivity pneumonitis?

A

Progressive breathlessness
Progressive cough

66
Q

What are some clinical signs of chronic hypersensitivity pneumonitis?

A

Possible crackles
Finger clubbing (Unusual)
Upper zone pulmonary fibrosis on CXR
Restrictive pattern on PFTs

67
Q

How is hypersensitivity pneumonitis diagnosed?

A

By identifying exposure using precipitins (IgG antibodies) to certain antigens and lung biopsy if in doubt

68
Q

What are the treatment options for hypersensitivity pneumonitis?

A

Removal of stimulus
Oral steroids if breathless or low gas transfer

69
Q

What is the old name for idiopathic pulmonary fibrosis?

A

Cryptogenic fibrosing alveolitis

70
Q

What is meant by idiopathic pulmonary fibrosis?

A

Pulmonary scarring with no identifiable cause

71
Q

What is the suspected pathophysiology of idiopathic pulmonary fibrosis?

A

It is thought to be caused by an imbalance in the fibrotic repair system, causing increased laying down of collagen fibres by fibroblasts

72
Q

Who is more at risk of idiopathic pulmonary fibrosis?

A

Men who smoke

73
Q

What are some conditions that can increase the risk of idiopathic pulmonary fibrosis?

A

Rheumatoid arthritis
Systemic lupus erythematosus
Systemic sclerosis
Asbestosis

74
Q

What are some drugs that can cause idiopathic pulmonary fibrosis?

A

Amiodarone
Busulphan
Bleomycin
Penicillamine
Nitrofurantoin
Methotrexate
Hydralazine

75
Q

What are some possible symptoms of idiopathic pulmonary fibrosis?

A

Progressive breathlessness over several years
Dry cough

76
Q

What are some possible clinical signs of idiopathic pulmonary fibrosis?

A

Finger clubbing
Bilateral fine inspiratory crackles
Restrictive defect on PFT
Bilateral infiltrates on CXR

77
Q

How does pulmonary fibrosis show on CT pulmonary angiogram?

A

“Honeycombing”
Reticulonodular shadowing worse at lung bases and periphery
Traction bronchiectasis

78
Q

What are some tests that can be used in the diagnosis of idiopathic pulmonary fibrosis?

A

PFTs -> Restrictive pattern
CXR -> Loss of definition of borders
CT pulmonary angiogram

79
Q

What are some conditions that need to be ruled out before making a diagnosis of idiopathic pulmonary fibrosis?

A

Occupational disease
Connective tissue disease - Rheumatoid arthritis, scleroderma, Sjögren’s disease, SLE
Left ventricular failure
Sarcoidosis
Hypersensitivity pneumonitis

80
Q

Describe the pathology of idiopathic pulmonary fibrosis?

A

Presence of an unusual interstitial pneumonia pattern ,with heterogenous fibrosis in alveolar walls, with fibrotic foci and destruction of architecture, with very little inflammation

81
Q

What are some pharmacological treatment options in idiopathic pulmonary fibrosis?

A

Oral steroids
Immunosupressants
Anti-fibrotics (Prevent further fibrosis)

82
Q

What are some examples of anti-fibrotic used in the treatment of idiopathic pulmonary fibrosis?

A

Pirfenidone
Nintedanib

83
Q

What are some possible side effects of pirfenidone and nintedanib?

A

Decreased appetite
Photosensitivity

84
Q

What is a possible surgical intervention in idiopathic pulmonary fibrosis?

A

Lung transplant in younger patients

85
Q

What are pneumoconioses?

A

These are a group of interstitial lung diseases caused by the inhalation of environmental particles, usually occupational

86
Q

What are the 2 main classes of pneumoconiosis?

A

Simple
Complicated

87
Q

What is meant by a simple pneumoconiosis?

A

Pneumoconiosis resulting in a chest x-ray abnormality only, with no symptoms present and no impairment of lung function

88
Q

How will pneumoconiosis appear on X-ray?

A

“Egg shell” opacification

89
Q

What is meant by complicated pneumoconiosis?

A

A symptomatic form of pneumoconiosis, characterised by progressive, massive fibrosis and increasing breathlessness, alongside the X-ray findings of simple pneumoconiosis

90
Q

What infection is a patient with complicated pneumoconiosis at an increased risk of contracting?

A

Tuberculosis

91
Q

What is Caplan’s syndrome?

A

This is a form of rheumatoid pneumoconiosis in which inhalation of occupational particles also mounts an inflammatory response

92
Q

Inhalation of what particulate causes Coal worker’s pneumoconiosis?

A

Coal dust

93
Q

Inhalation of what particulate causes Silicosis?

A

Silica dust or quartz

94
Q

Inhalation of what particulate causes Baritosis?

A

Barium

95
Q

Inhalation of what particulate causes Bird worker’s pneumoconiosis?

A

Avian proteins

96
Q

Inhalation of what particulate causes asbestosis?

A

Asbestos

97
Q

Inhalation of what particulate causes Hot tub lung (HP)?

A

Mycobacterium avium

98
Q

Inhalation of what particulate causes Coffee bean worker’s pneumoconiosis?

A

Coffee bean proteins

99
Q

Inhalation of what particulate causes Tobacco worker’s lung (HP)?

A

Aspergillus sp

100
Q

What occupations are more at risk of silicosis?

A

Miners
Foundry workers
Glass workers
Boiler workers

101
Q

What occupation is most at risk of baritosis?

A

Barium miners

102
Q

What are the main uses of barium?

A

Radiology
Industry

103
Q

How does baritosis show on chest x-ray?

A

Barium is highly radiopaque so will show a large, white mass on x-ray

104
Q

What are the main uses of asbestos?

A

Mining
Construction
Ship building
Boilers
Piping
Automotive components such as break linings

105
Q

What are the 3 types of asbestos?

A

Chrysotile - White asbestos
Amosite - Brown asbestos
Crocidolite - Most dangerous

106
Q

How much exposure to asbestos is required for asbestosis to form?

A

At least 5 years of prolonged exposure

107
Q

What are some common mucus and lung biopsy findings in asbestosis?

A

Asbestos bodies in lung tissue and sputum

108
Q

What are the 4 main asbestos related pleural diseases?

A

Benign pleural plaques
Acute asbestos pleuritis
Pleural effusion and diffuse pleural thickening
Malignant mesothelioma

109
Q

What causes the formation of benign pleural plaques?

A

Inhalation of asbestos activates macrophages, which cannot break it down
This causes asbestos fibres to migrate to the lung surface, therefore aggravating the pleural surface and causing plaque formation

110
Q

What are some possible symptoms of acute asbestos pleuritis?

A

Inflammation of the pleura can lead to:
Fever
Pain
Blood pleural effusion

111
Q

In who does asbestos increase the risk of bronchial carcinoma?

A

Smokers

Respiratory (23 decks)

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