Interstitial Lung Disease Flashcards Preview

ESA 3 - Respiratory System > Interstitial Lung Disease > Flashcards

Flashcards in Interstitial Lung Disease Deck (159)
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1
Q

What is the interstitial space?

A

A potential space between alveolar cells and the capillary basement membrane, which is only apparent in disease states

2
Q

What may the interstitial space contain in disease states?

A

Fibrous tissue, cells, or fluid

3
Q

What is interstitial lung disease?

A

A group of diseases with a variety of causes, that all have similar pathological effects and clinical features

4
Q

What is the pathophysiology of interstitial lung disease?

A

The development of fibrous tissue in the intersticium, producing a restrictive ventilatory defect

5
Q

Why does the development of fibrous tissue in the intersticium produce a restrictive ventilatory defect?

A

Because it makes the lungs less compliant

6
Q

Is airway resistance increased in interstitial lung disease?

A

No

7
Q

How can the FEV1/FVC ratio be kept >70% in interstitial lung disease?

A

Due to increased radial traction on the airway keeping the airway open

8
Q

What impairs gas exchange in interstitial lung disease?

A

Lengthening of the diffusion path

9
Q

What is selectively affected in interstitial lung disease?

A

Oxygen uptake

10
Q

Why is oxygen uptake selectively affected in interstitial lung disease?

A

As CO2 diffuses much more readily

11
Q

What are the symptoms of interstitial lung disease?

A
  • Shortness of breath
  • Reduced exercise tolerance
  • Dry cough
12
Q

What are the signs of interstitial lung disease?

A
  • Tachypnoea
  • Tachycardia
  • Reduced chest movement bilaterally
  • Coarse crackles
13
Q

What signs may be present in interstitial lung disease?

A
  • Cyansosis
  • Signs of right heart failure
14
Q

When is clubbing seen in interstitial lung disease?

A

Cryptogenic fibrosing alveolitis

15
Q

What are the categories of causes of interstitial lung disease?

A
  • Occupational
  • Treatment related
  • Connective tissue disease
  • Immunological
  • Idiopathic
16
Q

What are the occupational causes of interstitial lung disease?

A
  • Abestosis
  • Silicosis
  • Coal workers pneumoconiosis
17
Q

What are the treatment related causes of interstitial lung disease?

A
  • Radiation
  • Methotrexate
  • Nitrofurantoin
  • Amiodarone
  • Chemotherapy
18
Q

What connective tissue diseases can cause interstitial lung disease?

A
  • Rheumatoid arthritis
  • SLE
  • Polymyositis
  • Schleroderma
  • Sjorgen’s
19
Q

What are the immunological causes of interstitial lung disease?

A
  • Sarcoidosis
  • Hypersensitivity pneumonitis (EAA)
20
Q

What are the idiopathic causes of interstitial lung disease?

A
  • CFA/IPF
  • UIP/NSIP
  • DIP
  • LIP
  • RB-ILD
  • COP (BOOP)

CFA- cryptogenic fibrosing alveolitis
IPF- idiopathic pulmonary fibrosis
UIP- usual interstitial pneumonia
NSIP- non-specific interstial pnuemonia
DIP- desquamative interstial pneumonia
LIP- lymphoid interstitial pnuemonia
RBILD- respiratory bronchiolitis interstiial lung disease
COP- crytogenic organising pneumonia
BOOP- bronchiolitis oblitirans organising pneuomnia

for princess elliot

21
Q

What is fibrosing alveolitis?

A

A progressive inflammatory condition of an unknown cause

22
Q

How common is fibrosing alveolitis?

A

Relatively rare, 3-5 cases per 100,000

23
Q

How does the incidence of fibrosing alveolitis differ between the sexes?

A

It is two times more common in males

24
Q

What are the histological features of fibrosing alveolitis?

A

Increased activated alveolar macrophages

25
Q

What is the effect of increased activated alveolar macrophages in fibrosing alveolitis?

A
  • Attract neutrophils and eosinophils
  • Local lung damage
  • Tissue destruction and fibrosis
26
Q

Why do increased alveolar macrophages lead to local lung damage?

A

Due to ROS and proteases

27
Q

What do patients report with fibrosing alveolitis?

A

Progressive shortness of breath on exercise, often with non-productive cough

28
Q

Do patients with fibrosing alveolitis have finger clubbing?

A

Most do

29
Q

What does the chest x-ray show in patients with fibrosing alveolitis?

A

Small lungs with micro-nodular shadowing predominating in the lower lobes, with ragged heart borders

30
Q

When can fibrosing alveolitis be restrained?

A

In the early stages, less effective once fibrosis has developed

31
Q

How can fibrosing alveolitis be restrained?

A

By treatment with high dose oral steroids

32
Q

How is the effectiveness of treatment for fibrosing alveolitis monitored?

A

By repeated lung function tests

33
Q

What causes extrinsic allergic alveolitis?

A

Inhalation of organic material triggers an allergic reaction in alveoli and bronchioles

34
Q

Is extrinsic allergic alveolitis an acute or chronic condition?

A

Can be either

35
Q

What are the characteristics of acute extrinsic allergic alveolitis?

A
  • Sudden onset
  • Rapidly progressing
36
Q

Give an example of acute extrinsic allergic alveolitis

A

Farmer’s Lung

37
Q

What is the antigen in Farmer’s Lung?

A

Thermophillic actinomycetes found in mouldy hay

38
Q

How does Farmer’s Lung present?

A
  • Influenza like illness 4-9 hours layer with a dry cough and breathlessness on exertion
  • Fine mid and late inspiratory crackles
  • May be a wheeze
39
Q

Give an example of chronic extrinsic allergic alveolitis

A

Bird Fancier’s Lung

40
Q

What causes Bird Fancier’s Lung?

A

Long term antigen exposure from pigeons and budgerigars

41
Q

How does Bird Fancier’s lung present?

A
  • Insidious malaise
  • Dry cough and breathlessness over months and years
  • Inspiratory crackles
42
Q

What is meant by insidious malaise?

A

Feeling particularly unwell

43
Q

Does finger clubbing occur with extrinsic allergic alveolitis?

A

No

44
Q

What does a chest x-ray show in acute extrinsic allergic alveolitis?

A

Diffuse micro-nodular infiltrate, denser towards the hila

45
Q

What does a chest x-ray show in chronic extrinsic allergic alveolitis?

A

May be almost normal, progressing to fibrosis in late disease

46
Q

What do lung function tests show in extrinsic allergic alveolitis?

A
  • Reduced compliance
  • Reduced gas transfer
47
Q

What causes asbestosis?

A

Inhalation of asbestos fibres

48
Q

When does asbestosis often develop?

A

Long after the exposure

49
Q

What is asbestos inhalation associated with?

A
  • Three forms of disease
    • Benign pleural plaques
    • Asbestosis (pulmonary fibrosis)
    • Mesothelioma
  • Marked increase in lung cancer
50
Q

What do asbestos fibres produce alveolitis?

A

When they can penetrate the alveoli

51
Q

What happens once asbestos fibres have produced alveolitis?

A
  • There is an influx of macrophages
  • Alveolitis progresses to fibrosis
52
Q

What does the influx of macrophages produce in alveolitis caused by asbestos?

A

Characteristic asbestosis bodies

53
Q

What is required for a diagnosis of asbestosis?

A

A history of asbestos exposure

54
Q

How does a patient with asbestosis present?

A
  • Breathless on exertion
  • Dry cough
  • Inspiratory crackles at lung bases, which rise as the disease advances
55
Q

What is the treatment for asbestosis?

A

None

56
Q

What do lung function tests show in a patient with asbestosis?

A
  • Small lungs
  • Reduced compliance
  • Impaired gas transfer
57
Q

What causes sarcoidosis?

A

Unknown

58
Q

What is sarcoidosis characterised by?

A

Non-caseating granulomas (non-necrotising) in multiple organs and body sites

59
Q

Where is sarcoidosis most common?

A

In the lungs

60
Q

What happens in sarcoidosis?

A
  • Fluid is collected by lavage in the airways
  • Alveoli contain lots of cells
61
Q

What cells are present in large numbers in the alveoli in sarcoidosis?

A

Macrophages and lymphocytes

62
Q

What is the epidemiology of sarcoidosis?

A
  • More common in Afro-Caribbean and Asians than Caucasians
  • Highest incidence in 30’s and 40’s
  • More female cases
63
Q

What can be determined from the fact that there is a difference in incidence between races?

A

There is a genetic predisposition

64
Q

How does sarcoidosis present?

A
  • Often asymptomatic
  • Cough
  • Breathlessness
65
Q

How is the severity of sarcoidosis assessed?

A

Grading system from 0-4

66
Q

What do chest x-rays show in patients with sarcoidosis?

A
  • Miliary and nodular shadowing
  • Diffuse fibrosis
67
Q

What is the treatment for sarcoidosis?

A

Steroids

68
Q

When are steroids effective in supressing sarcoidosis?

A

Stages 1-3

69
Q

Give 4 occupational lung diseases

A
  • Asthma
  • Diffuse fibrosis
  • Nodular fibrosis (e.g. pneumoconiosis)
  • Alveolitis
70
Q

What occupation is asthma associated with?

A

Lab workers

71
Q

What is asthma related to being a lab worker due to exposure to?

A

Rat urine

72
Q

What occupation is diffuse fibrosis associated with?

A
  • Boiler/pipe laggers
  • Railway/construction
73
Q

What is diffuse fibrosis associated with exposure to?

A

Asbestos

74
Q

What occupations is nodular fibrosis associated with?

A
  • Coal Miner
  • Miner
  • Demolition
75
Q

What is nodular fibrosis associated withe exposure to?

A
  • Coal dust
  • Silica
  • Asbestos
76
Q

What occupations is alveolitis associated with?

A
  • Farmer
  • Pigeon Fancier
77
Q

What is alveolitis associated with exposure to?

A
  • Fungal spores from hay
  • Avian antigens
78
Q

What is the x-ray appearance of fibrosing alveolitis?

A
  • Small lungs
  • Micro-nodular shadowing (lower lobes)
  • Ragged heart borders
79
Q

What is the x-ray appearance of acute extrinsic allergic alveolitis?

A
  • Micro-nodular infiltrate
  • Denser towards hila
80
Q

What is the x-ray appearance of chronic extrinsic allergic alveolitis?

A
  • Almost normal
  • Progresses to fibrosis in late disease
81
Q

What is the x-ray appearance of sarcoidosis?

A
  • Miliary and nodular shadowing
  • Diffuse fibrosis
82
Q

What is the x-ray appearance of asbestosis?

A
  • Holly leaf plaques
  • Fibrosis
  • Mesothelioma
83
Q

What is the pleura?

A

A serous membrane

84
Q

What does the pleura consist of?

A

A single layer of mesothelial cells with a thin layer of underlying connective tissue

85
Q

Waht does the parietal pleura line?

A

The inside of each hemi thorax

86
Q

What does each hemi thorax consist of?

A
  • The bony thoracic cage
  • Diaphragm
  • Mediastinal surface
87
Q

What does the parietal pleura become continuous with?

A

The visceral pleura

88
Q

Where does the parietal pleura become continuous with the visceral pleura?

A

At the hilum of the lung

89
Q

Where does the visceral pleura extend?

A

Between lobes of the lung into the depths of the oblique and horizonal fissures

90
Q

What is the pleural cavity?

A

A potential space between the two layers of pleura that are continuous at the hilum

91
Q

What are both layers of pleura covered with?

A

A common film of fluid

92
Q

What produces the common film of fluid covering the layers of pleura?

A

The parietal surface

93
Q

What is the fluid produced from the parietal surface absorbed by?

A

Parietal lymphatic vessels

94
Q

What does the pleural fluid allow?

A

The two layers to slide on one another, thus in heatlh the pleura allows movement of the lung against the chest wall while breathing

95
Q

What does the surface tension of the pleural fluid provide?

A

The cohesion that keeps the lung surface in contact with the thoracic wall

96
Q

What is the result of the lung surface being in contact with the thoracic wall?

A

When the thorax expands in inspiration, the lung expands along with it and fills with air

97
Q

Do the lungs occupy all the available space in the pleural cavity?

A

No, even in deep inspiration

98
Q

Label this diagram

A
  • A - Pleural cavity
  • B - Collapsed lung
  • C - Visceral pleura
  • D - Parietal pleura
  • E - Thoracic wall lined with endothoracic fascia
  • F - Pheno-pleural fascia (part of endothoracic fascia)
  • G - Suprapleural membrane
  • H - Parts of parietal pleura
    • Hi - Cervical pleura
    • Hii - Mediastinal part
    • Hiii - Costal part
    • Hiv - Diaphragmatic part
  • I - Endothoracic fascia
  • J - Visceral pleura
  • K - Diaphragm
  • L - Mediastinum (contains heart)
  • Orange - Visceral pleura
  • Purple - Parietal pleura
  • Blue - Costal pleura
  • Green - Diaphragmatic pleura
  • Red - Mediastinal pleura
  • Yellow - Cervical pleura
  • Solid - Fascial membrane (Suprapleural and mediastinal fascia, fibrous pericardium)
  • Dashed - Endothoracic fascia
99
Q

How much pleural fluid is turned over per day?

A

15ml, but can increase to 300ml

100
Q

What produces pleural fluid?

A

Capillary filtration at the parietal pleura

101
Q

How does capillary filtration produce pleural fluid?

A

Starling Forces

102
Q

When does the amount of pleural fluid increase?

A
  • When theres an increase in lung interstitial fluid
  • When theres an increase in hydrostatic pressure
  • When theres a increase in permeability
  • When theres a decrease in oncotic pressure
103
Q

What condition may cause an increase in hydrostatic pressure?

A

Heart failure

104
Q

What conditions may cause an increase in permeability?

A
  • Inflammation
  • Sepsis
  • Malignancy
105
Q

What condition may cause a decrease in oncotic pressure?

A

Liver failure

106
Q

How is pleural fluid absorbed?

A

Via lymphatic drainage

107
Q

What can decrease the rate of lymphatic drainage of pleural fluid?

A

Lymphatic blockage

108
Q

What can increase the rate of lymphatic drainage?

A

Increase in systemic venous pressure

109
Q

What is a pleural effusion?

A

Any collection of extra fluid in the pleural space

110
Q

What fluids can collect in the pleural space?

A
  • Blood
  • Chyle
  • Pus
  • Serous fluid
111
Q

What is chyle?

A

Lymph with fats in it

112
Q

What is a pleural effusion with blood called?

A

Hemothorax

113
Q

What is a pleural effusion with chyle called?

A

Chylothorax

114
Q

What is a pleural effusion with pus called?

A

Empyema

115
Q

What is a pleural effusion with serous fluid called?

A

Simple effusion

116
Q

What are simple pleural effusions characterised by?

A

Protein content

117
Q

What do transudates have?

A

Low protein content

118
Q

What is the protein content of transudates?

A

<30g/L

119
Q

What do exudates have?

A

High protein content

120
Q

What is the protein content of exudates?

A

>30g/L

121
Q

What can cause transudates?

A
  • Increased hydrostatic pressure
  • Decreased capillary oncotic pressure
  • Increased capillary pressure
122
Q

What condition can cause increased hydrostatic pressure?

A

Cardiac failure

123
Q

What conditions can cause decreased capillary oncotic pressure?

A
  • Hypoalbuminaemia
  • Nephrotic syndrome
124
Q

What condition can cause increased capillary permeability?

A

Sepsis

125
Q

What can cause exudates?

A
  • Neoplasms
  • Infection
  • Immune disease
  • Abdominal disease
126
Q

What neoplasms can cause exudates?

A
  • Cancer involving pleural surface
  • Secondary’s from breast, lung, ovarian, GI, lymphoma
  • Primary tumour of pleura
127
Q

Give two infections that can cause exudates

A
  • Pneumonia
  • TB
128
Q

What immune diseases can cause exudates?

A

Connective tissue diseases

129
Q

Give two examples of connective tissue immune diseases that can cause exudates

A
  • RA
  • SLE
130
Q

Give 3 abdominal diseases that can cause exudates

A
  • Pancreatitis
  • Ascites
  • Subphrenic abscess
131
Q

How does pancreatitis cause exudates?

A

Diaphragmatic inflammation

132
Q

How does ascites cause exudates?

A

When it transverses the diaphragm

133
Q

What is pleurisy, or pleuritis?

A

Inflammation of the pleura

134
Q

What are the symptoms of pleurisy?

A
  • Sharp pain on inspiration
  • Pain worse on coughing, sneezing, laughing, etc.
  • Patients take small breaths, and hold affected side of chest
  • Pain on shoulder on the same side
  • Pleural rub
135
Q

Why does pleurisy produce pain in the shoulder on the same side?

A

Because of involvement of the diaphragmatic pleura

136
Q

What is meant by pleural rub?

A

A creaking noise heard through a stethoscope with respiratory movements

137
Q

What are the causes of pleurisy?

A
  • Infection
  • Autoimmune
    Lung cancer
  • Pneumothorax
  • Pulmonary embolism
138
Q

What is the most common cause of pleurisy?

A

Infection

139
Q

What infections can cause pleurisy?

A
  • TB
  • Pneumonia
140
Q

What autoimmune conditions can cause pleurisy?

A
  • SLE
  • RA
141
Q

What may lead to pleural fibrosis?

A

Unabsorbed pleural effusion

142
Q

What is the effect of pleural fibrosis?

A
  • A small degree of thickening has no effects
  • Wide spread fibrosis restricts expansion, with a measurable reduction in lung volumes and compliance
143
Q

Are secondary deposits of tumours uncommon in the pleura?

A

No

144
Q

What is the most common primary tumour of the pleura?

A

Malignany mesothelioma

145
Q

What is true of almost all victims of malignant mesothelioma?

A

They were exposed to asbestos 20-40 years before

146
Q

What are the early symptoms of malignant mesothelioma?

A

Those of a pleural effusion, but with a duller pain

147
Q

What are the signs of a malignant mesothelioma?

A

That of a large pleural effusion

148
Q

What are the types of chest wall abnormalities?

A
  • Deformation of the ribs, sternum, and thoracic spine
  • Acquired abnormalities
149
Q

Give two examples of sternal abnormalities

A
  • Pectus Carcinatum
  • Pectus excavatum
150
Q

What do sternal abnormalities produce?

A

Cosmetic deformities, rarely produce functional impairment

151
Q

What may scoliosis and kyphosis produce?

A

Significant functional impairment of the thoracic cage

152
Q

How can chest wall abnormalities be acquired?

A
  • Trauma producing broken ribs
  • Surgery for TB
153
Q

What may trauma producing broken ribs cause?

A

Possible pneumothorax

154
Q

What is the purpose of surgery for TB?

A

Designed to collapse their lung

155
Q

Who may have had surgery for TB?

A

Old patients

156
Q

How may the muscles involved in breathing be affected?

A
  • By generalised muscular diseases
  • Neurological diseases
157
Q

What generalised muscular disease can affect the muscles involved in breathing?

A

Muscular dystrophy

158
Q

What neurological diseases can affect the muscles involved in breathing?

A
  • Motor neurone disease
  • Polio
159
Q

What does weakness of muscles involved in breathing produce?

A

Respiratory failure with lower resistance to respiratory tract infections because of poor clearance of secretions