Interstitial Lung Disease Flashcards

1
Q

What medication is most commonly used to try to prevent progression of pulmonary fibrosis?

A

High dose corticosteroids

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2
Q

What is the only definitive treatment option for pulmonary fibrosis, and who could receive it?

A

Lung transplant- given to patients aged < 65 with severe, progressive disease

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3
Q

What are some interventions that may help to relieve symptoms of pulmonary fibrosis?

A

Pulmonary rehabilitation and oxygen therapy

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4
Q

What is the most common type of hypersensitivity pneumonitis in the UK?

A

Bird fancier’s lung

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5
Q

What is the relationship between smoking and hypersensitivity pneumonitis?

A

Smoking reduces the risk of developing hypersensitivity pneumonitis

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6
Q

Where in the lungs is pulmonary fibrosis caused by hypersensitivity pneumonitis most commonly found?

A

The upper lobes

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7
Q

What are the three main features of interstitial lung disease?

A

Progressive breathlessness, restrictive lung function pattern, reduced transfer factor

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8
Q

The diagnosis of ILD mainly depends on the use of what investigation?

A

High resolution CT

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9
Q

What are the two most common interstitial lung diseases?

A

Idiopathic pulmonary fibrosis and sarcoidosis

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10
Q

Name some potential causes of pulmonary fibrosis?

A

Idiopathic, connective tissue disease, drugs, radiotherapy, ARDS

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11
Q

What blood test should be done to rule out connective tissue disease as a cause of pulmonary fibrosis?

A

Autoantibody screen

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12
Q

What blood test should be done to rule out hypersensitivity pneumonitis as a cause of pulmonary fibrosis?

A

Avian precipitins

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13
Q

What blood test should be done to rule out sarcoidosis as a cause of pulmonary fibrosis?

A

Serum ACE

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14
Q

What will a high resolution CT of pulmonary fibrosis show?

A

A ground-glass appearance and honeycombing

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15
Q

What further investigation can be used in those with suspected pulmonary fibrosis to exclude other diagnoses?

A

Bronchoscopy and bronchoalveolar lavage

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16
Q

What are the three main areas of management for pulmonary fibrosis?

A

Treat any underlying cause, advise smoking cessation, prevent progression with medical treatment

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17
Q

Give some examples of drugs which can potentially cause pulmonary fibrosis?

A

Chemotherapy agents, DMARDs and amiodarone

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18
Q

Name some risk factors for the development of pulmonary fibrosis?

A

Male, increased age, smoking, occupational exposure to fumes and dusts

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19
Q

What is the median age for presentation with idiopathic pulmonary fibrosis vs. pulmonary fibrosis associated with connective tissue disease?

A

70 years for idiopathic, 50-60 years for CTD

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20
Q

What happens to the total lung volume in pulmonary fibrosis?

A

Decreased

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21
Q

What happens to the FVC in pulmonary fibrosis?

A

Decreased

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22
Q

What happens to FEV1 in pulmonary fibrosis?

A

Decreased

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23
Q

What happens to the FEV1: FVC ratio in pulmonary fibrosis?

A

Normal (> 75%)

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24
Q

In sarcoidosis, affected tissues are infiltrated with what?

A

Non-caseating granulomas

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25
Q

Describe the typical demographic of an individual affected by sarcoidosis?

A

Female, 20-40 years, Afro-Caribbean ethnicity

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26
Q

What type of hypersensitivity reaction is sarcoidosis?

A

Type IV

27
Q

Describe the four main features of acute sarcoidosis?

A

Bilateral hilar lymphadenopathy, fever, arthralgia, erythema nodosum

28
Q

What respiratory symptoms can be seen in sarcoidosis?

A

Dry cough and dyspnoea

29
Q

Eventually, progressive sarcoidosis leads to what respiratory change?

A

Pulmonary fibrosis

30
Q

Which electrolyte abnormality is often associated with sarcoidosis?

A

Hypercalcaemia

31
Q

What is the definitive investigation for diagnosing sarcoidosis?

A

Tissue biopsy

32
Q

An increased level of what can be detected in the blood of people with sarcoidosis, and correlates with disease activity?

A

Serum ACE

33
Q

Other than serum ACE, what are some other blood tests which may be increased in sarcoidosis?

A

Inflammatory markers, calcium, LFTs

34
Q

Name three features which may be seen on a CXR of sarcoidosis?

A

Hilar lymphadenopathy, pulmonary infiltrates, pulmonary fibrosis

35
Q

What imaging investigation is necessary for a diagnosis of sarcoidosis?

A

High resolution CT

36
Q

Other than sarcoidosis, what are the differentials of hilar lymphadenopathy?

A

TB and lymphoma

37
Q

Which individuals would require treatment for sarcoidosis?

A

Progressive lung disease and other end-organ involvement and hypercalcaemia

38
Q

What treatment options are available for sarcoidosis?

A

Systemic corticosteroids, hydroxychloroquine, methotrexate/infliximab

39
Q

Within what timeframe does acute sarcoidosis usually resolve?

A

2 years

40
Q

The pulmonary fibrosis associated with sarcoidosis typically affects where in the lungs?

A

Upper/mid zones

41
Q

Which type of pneumoconiosis may lead to the production of black sputum?

A

Coal worker’s pneumoconiosis

42
Q

What is Caplan’s syndrome?

A

Coal worker’s pneumoconiosis associated with rheumatoid arthritis

43
Q

Which type of pneumoconiosis may be associated with ‘eggshell’ calcification on CXR?

A

Silicosis

44
Q

Having silicosis increases an individual’s risk of developing which other condition?

A

Pulmonary TB

45
Q

What is pneumoconiosis?

A

Lung damage caused by inhalation of mineral dusts

46
Q

In pneumoconiosis, there is usually a history of what?

A

Occupational exposure

47
Q

Name the three most common types of pneumoconiosis seen in the UK?

A

Coal worker’s pneumoconiosis, asbestosis, silicosis

48
Q

What are some factors that may help to distinguish asbestosis from idiopathic pulmonary fibrosis?

A

Younger age of onset, presence of pleural plaques and asbestos bodies

49
Q

Are there any treatment options for pneumoconiosis?

A

No

50
Q

Describe the symptoms experienced in chronic hypersensitivity pneumonitis?

A

Progressive dyspnoea and dry cough, associated with weight loss and fatigue

51
Q

What happens to the transfer factor in hypersensitivity pneumonitis?

A

Reduced

52
Q

What is the treatment for hypersensitivity pneumonitis?

A

Allergen avoidance and high dose corticosteroids

53
Q

Describe the progression of chronic hypersensitivity pneumonitis, if allergen avoidance is not adhered to?

A

Progression to end-stage pulmonary fibrosis

54
Q

What types of hypersensitivity reaction can hypersensitivity pneumonitis be caused by?

A

Type III or type IV

55
Q

How soon after exposure to the antigen does acute hypersensitivity pneumonitis occur?

A

4-6 hours

56
Q

How quickly does acute hypersensitivity pneumonitis resolve?

A

Within 48-72 hours

57
Q

Describe the symptoms of acute hypersensitivity pneumonitis?

A

Episodic breathlessness, cough, wheeze and fever

58
Q

What pattern would pulmonary function tests show in hypersensitivity pneumonitis?

A

Either a restrictive, or mixed restrictive/obstructive pattern

59
Q

Describe the typical clinical presentation of pulmonary fibrosis?

A

Progressive dyspnoea on exertion and a dry cough

60
Q

Describe what happens to O2 saturations in early stages of pulmonary fibrosis?

A

Normal at rest but decrease on exercise

61
Q

Name some clinical signs of idiopathic pulmonary fibrosis?

A

Finger clubbing, reduced chest expansion, fine, inspiratory bibasal crackles

62
Q

What investigations should be done in someone presenting with features of pulmonary fibrosis?

A

Blood tests, PFTs, CXR and high-resolution CT

63
Q

What will a CXR of idiopathic pulmonary fibrosis show?

A

Bibasal reticulonodular shadowing