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
Q

What are examples of extrinsic allergic alveolitis?

A

Bird fancier’s lung, farmer’s lung

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26
Q

How would you describe a chronic case of extrinsic allergic alveolitis?

A

Extensive fibrosis with honeycombing and air-trapping

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27
Q

What are examples of pneumoconiosis?

A

Asbestosis, silicosis, coal worker’s lung

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28
Q

What type of lung disease is asbestosis?

A

Fibrotic

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29
Q

What colours can asbestos be and which is the worst?

A

Blue, brown, white- blue is worst

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30
Q

What is mesothelioma and when can it occur?

A

Malignant thickening of the pleura which can occur 20-40 years after asbestos exposure

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31
Q

Asbestos exposure combined with what makes a diagnosis even worse?

A

Smoking

32
Q

What are some effects of traffic fumes?

A

Reduced lung growth in children and increased rates of asthma and COPD

33
Q

What effects can many inhaled substances also have outside the lung?

A

Low birth weight, appendicitis, stroke, neurological outcomes

34
Q

What is sarcoidosis?

A

Multi-system granulomatous type IV hypersensitivity of unknown cause

35
Q

What type of granulomas are involved in sarcoidosis?

A

Non-caseating

36
Q

Who is sarcoidosis less common in?

A

Smokers

37
Q

What will acute sarcoidosis cause?

A

Erythema nodosum, bilateral hilar lymphadenopathy, arthritis and fever

38
Q

What does chronic sarcoidosis cause?

A

Lung and skin infiltrates, peripheral lymphadenopathy and hypercalcaemia

39
Q

What are some differentials of sarcoidosis?

A

TB, lymphoma, carcinoma, fungal infection

40
Q

How is sarcoidosis diagnosed?

A

CXR, tissue biopsy, pulmonary function tests, bloods

41
Q

What will a CXR of sarcoidosis cause?

A

Peripheral nodular infiltrate

42
Q

What will a tissue biopsy of sarcoidosis show?

A

Non-caseating granulomas

43
Q

What will pulmonary function tests for sarcoidosis show?

A

Restrictive pattern

44
Q

What will blood tests for sarcoidosis show?

A

Increased calcium and inflammatory markers

45
Q

What is the treatment for acute sarcoidosis?

A

Self limiting

46
Q

What is the treatment for chronic sarcoidosis?

A

Oral steroids or immunosuppression if a major organ is involved

47
Q

What is extrinsic allergic alveolitis/hypersensitivity pneumonitis?

A

Type III reaction to antigen lymphocyte alveolitis

48
Q

How will acute extrinsic allergic alveolitis present?

A

A few hours after antigen exposure with dry cough, SOB, myalgia and fever

49
Q

What signs may be present in acute extrinsic allergic alveolitis?

A

Crackles but no wheeze, hypoxia

50
Q

How do you treat acute extrinsic allergic alveolitis?

A

Oxygen, steroids and antigen avoidance

51
Q

What does chronic extrinsic allergic alveolitis cause?

A

Dry cough, progressive breathlessness, crackles

52
Q

Where is the fibrosis most common in extrinsic allergic alveolitis?

A

Upper zones

53
Q

What may be increased in extrinsic allergic alveolitis?

A

IgG

54
Q

How do you treat chronic extrinsic allergic alveolitis?

A

Allergen avoidance and maybe oral steroids

55
Q

Though no direct cause is known, what are some secondary causes of idiopathic pulmonary fibrosis?

A

Smoking, rheumatoid, SLE, sclerosis, drugs, asbestos

56
Q

How does idiopathic pulmonary fibrosis present?

A

Progressive breathlessness and dry cough, clubbing and bibasal fine inspiratory crackles with decreased lung volumes

57
Q

What will a CT of idiopathic pulmonary fibrosis show?

A

Reticulonodular shadowing worse at lung bases an peripheries

58
Q

What is the buzzword associated with idiopathic pulmonary fibrosis?

A

Ground glass appearance on CXR

59
Q

What are some differentials of idiopathic pulmonary fibrosis?

A

Occupational or connective tissue disease, LVF, extrinsic allergic alveolitis or sarcoidosis

60
Q

What is the treatment for idiopathic pulmonary fibrosis?

A

No treatment really- don’t give steroids or immunosuppressants. Can give oxygen if hypoxic or lung transplant if patient is young

61
Q

What is the prognosis for idiopathic pulmonary fibrosis?

A

Most progress to respiratory failure and live for 4/5 years after diagnosis

62
Q

What are normal findings of coal worker’s pneumoconiosis?

A

CXR abnormality with no impairment of lung function

63
Q

What will complicated pneumoconiosis show?

A

Massive fibrosis

64
Q

What is Caplan’s syndrome and what will it show?

A

Rheumatoid pneumoconiosis- pulmonary nodules

65
Q

What will show ‘egg shell calcification of hilar nodes’ on CXR?

A

Silicosis

66
Q

What are the symptoms of benign pleural plaques?

A

Asymptomatic

67
Q

What are symptoms of acute asbestos pleuritic?

A

Fever, pain and bloody pleural effusion

68
Q

What will mesothelioma present with and what is the treatment?

A

Chest pain and pleural effusion- no treatment

69
Q

What are type 1 alveolar cells?

A

Squamous epithelial cells which line most of the alveolar surface

70
Q

What are type 2 alveolar cells?

A

Squamous epithelial cells which make up the rest of the alveolar surface but are also secretory cells

71
Q

What exemplifies early stage acute ILD?

A

Adult respiratory distress syndrome

72
Q

What is fibrosis get alveolitis?

A

Subpleural and basal fibrosis with variable inflammatory components

73
Q

Are connective tissue diseases milder or harsher than fibrosis alveolitis?

A

Milder

74
Q

What can often accompany connective tissue diseases?

A

Pleural effusions and rheumatoid nodules

75
Q

What 4 things can asbestos cause?

A

Pleural plaques, interstitial fibrosis (asbestosis), bronchial carcinoma, mesothelioma