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Restrictive (Interstitial) Diseases Flashcards

1
Q

What causes outwith the lungs can cause an interstitial disease?

A

Skeletal, muscular or compression due to abdominal obesity or ascites

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2
Q

What muscles would have to be affected to cause interstitial lung disease?

A

Intercostals or diaphragm

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3
Q

What conditions can cause muscle damage leading to ILD?

A

Myasthenia gravis and Guillain Barre syndrome and motor neurone disease

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4
Q

What happens to physiological volumes as ILD causes chronic alveolar under-ventilation?

A

Decreased PaO2, increased PaCO2, decreased lung volumes

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5
Q

What can the timing of ILDs be?

A

Acute, episodic or chronic

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6
Q

What can internal causes of chronic IDL be generally?

A

Environmental or occupational agents, drugs, systemic disease, idiopathic

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7
Q

What is ILD do to disease of?

A

Alveolar structures

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8
Q

What are 3 of the biggest groups of causes of ILD?

A

Fluid in alveolar spaces, inflammatory tissues in alveolar spaces and inflammation in alveolar walls (alveolitis)

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9
Q

What can fluid in alveolar air spaces cause?

A

Pulmonary oedema or non-cardiac pulmonary oedema

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10
Q

What causes pulmonary oedema?

A

Increased pulmonary venous pressure

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11
Q

What causes non-cardiac pulmonary oedema?

A

Normal pulmonary venous pressure but leaky capillaries due to sepsis or trauma

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12
Q

What can inflammatory components in alveolar spaces cause? Give examples.

A

Consolidation (infective pneumonia), infarction (PE, vasculitis)

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13
Q

What can cause alveolitis?

A

Granulomatous, extrinsic allergic (i.e. from birds), sarcoidosis, drugs, fibrosing, autoimmune

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14
Q

What is sarcoidosis?

A

Multi system disorder causing lymphadenopathy

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15
Q

What are other causes of ILD?

A

Rheumatoid arthritis, drugs, idiopathic

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16
Q

What is an example of something causing eosinophilic alveolitis?

A

Aspergillus

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17
Q

What do clinical features of ILD include?

A

Cough but no wheeze, breathless on exertion, finger clubbing, inspiratory crackles, central cyanosis

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18
Q

What can later stage ILD cause?

A

Pulmonary fibrosis

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19
Q

What can help make a diagnosis of ILD?

A

History, decreased lung volumes but normal peak flow, low SpO2, CXR, ECHO, HRCT

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20
Q

What will a CXR of ILD show?

A

Bilateral diffuse alveolar infiltrates

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21
Q

Why is an ECHO done for ILD?

A

To exclude heart failure

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22
Q

What is the treatment for ILD?

A

Remove trigger and treat inflammation: 1st line systemic corticosteroids 2nd line azathioprine. Give oxygen if hypoxic.

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23
Q

What is the last resort treatment for ILD?

A

Lung transplant

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24
Q

What is another name for extrinsic allergic alveolitis?

A

Hypersensitivity pneumonitis

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25
What are examples of extrinsic allergic alveolitis?
Bird fancier's lung, farmer's lung
26
How would you describe a chronic case of extrinsic allergic alveolitis?
Extensive fibrosis with honeycombing and air-trapping
27
What are examples of pneumoconiosis?
Asbestosis, silicosis, coal worker's lung
28
What type of lung disease is asbestosis?
Fibrotic
29
What colours can asbestos be and which is the worst?
Blue, brown, white- blue is worst
30
What is mesothelioma and when can it occur?
Malignant thickening of the pleura which can occur 20-40 years after asbestos exposure
31
Asbestos exposure combined with what makes a diagnosis even worse?
Smoking
32
What are some effects of traffic fumes?
Reduced lung growth in children and increased rates of asthma and COPD
33
What effects can many inhaled substances also have outside the lung?
Low birth weight, appendicitis, stroke, neurological outcomes
34
What is sarcoidosis?
Multi-system granulomatous type IV hypersensitivity of unknown cause
35
What type of granulomas are involved in sarcoidosis?
Non-caseating
36
Who is sarcoidosis less common in?
Smokers
37
What will acute sarcoidosis cause?
Erythema nodosum, bilateral hilar lymphadenopathy, arthritis and fever
38
What does chronic sarcoidosis cause?
Lung and skin infiltrates, peripheral lymphadenopathy and hypercalcaemia
39
What are some differentials of sarcoidosis?
TB, lymphoma, carcinoma, fungal infection
40
How is sarcoidosis diagnosed?
CXR, tissue biopsy, pulmonary function tests, bloods
41
What will a CXR of sarcoidosis cause?
Peripheral nodular infiltrate
42
What will a tissue biopsy of sarcoidosis show?
Non-caseating granulomas
43
What will pulmonary function tests for sarcoidosis show?
Restrictive pattern
44
What will blood tests for sarcoidosis show?
Increased calcium and inflammatory markers
45
What is the treatment for acute sarcoidosis?
Self limiting
46
What is the treatment for chronic sarcoidosis?
Oral steroids or immunosuppression if a major organ is involved
47
What is extrinsic allergic alveolitis/hypersensitivity pneumonitis?
Type III reaction to antigen lymphocyte alveolitis
48
How will acute extrinsic allergic alveolitis present?
A few hours after antigen exposure with dry cough, SOB, myalgia and fever
49
What signs may be present in acute extrinsic allergic alveolitis?
Crackles but no wheeze, hypoxia
50
How do you treat acute extrinsic allergic alveolitis?
Oxygen, steroids and antigen avoidance
51
What does chronic extrinsic allergic alveolitis cause?
Dry cough, progressive breathlessness, crackles
52
Where is the fibrosis most common in extrinsic allergic alveolitis?
Upper zones
53
What may be increased in extrinsic allergic alveolitis?
IgG
54
How do you treat chronic extrinsic allergic alveolitis?
Allergen avoidance and maybe oral steroids
55
Though no direct cause is known, what are some secondary causes of idiopathic pulmonary fibrosis?
Smoking, rheumatoid, SLE, sclerosis, drugs, asbestos
56
How does idiopathic pulmonary fibrosis present?
Progressive breathlessness and dry cough, clubbing and bibasal fine inspiratory crackles with decreased lung volumes
57
What will a CT of idiopathic pulmonary fibrosis show?
Reticulonodular shadowing worse at lung bases an peripheries
58
What is the buzzword associated with idiopathic pulmonary fibrosis?
Ground glass appearance on CXR
59
What are some differentials of idiopathic pulmonary fibrosis?
Occupational or connective tissue disease, LVF, extrinsic allergic alveolitis or sarcoidosis
60
What is the treatment for idiopathic pulmonary fibrosis?
No treatment really- don't give steroids or immunosuppressants. Can give oxygen if hypoxic or lung transplant if patient is young
61
What is the prognosis for idiopathic pulmonary fibrosis?
Most progress to respiratory failure and live for 4/5 years after diagnosis
62
What are normal findings of coal worker's pneumoconiosis?
CXR abnormality with no impairment of lung function
63
What will complicated pneumoconiosis show?
Massive fibrosis
64
What is Caplan's syndrome and what will it show?
Rheumatoid pneumoconiosis- pulmonary nodules
65
What will show 'egg shell calcification of hilar nodes' on CXR?
Silicosis
66
What are the symptoms of benign pleural plaques?
Asymptomatic
67
What are symptoms of acute asbestos pleuritic?
Fever, pain and bloody pleural effusion
68
What will mesothelioma present with and what is the treatment?
Chest pain and pleural effusion- no treatment
69
What are type 1 alveolar cells?
Squamous epithelial cells which line most of the alveolar surface
70
What are type 2 alveolar cells?
Squamous epithelial cells which make up the rest of the alveolar surface but are also secretory cells
71
What exemplifies early stage acute ILD?
Adult respiratory distress syndrome
72
What is fibrosis get alveolitis?
Subpleural and basal fibrosis with variable inflammatory components
73
Are connective tissue diseases milder or harsher than fibrosis alveolitis?
Milder
74
What can often accompany connective tissue diseases?
Pleural effusions and rheumatoid nodules
75
What 4 things can asbestos cause?
Pleural plaques, interstitial fibrosis (asbestosis), bronchial carcinoma, mesothelioma

Respiratory (16 decks)

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