Interstitial and Occupational Lung Disease Flashcards

1
Q

What does interstitial disease interfere with and what is the lung pattern?

A

Interferes with gas transfer

Restrictive lung pattern

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2
Q

Give two symptoms of interstitial disease?

A

Breathlessness and dry cough

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3
Q

What class of interstitial lung disease is part of systemic disease, exposure to agent or idiopathic?

A

Chronic interstitial lung disease

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4
Q

Name two granulomatous interstitial lung diseases?

A

Sarcoidosis

Extrinsic allergic alveolitis

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5
Q

Name a granulomatous type 4 hypersensitivity disease of unknwon cause?

A

Sarcoidosis

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6
Q

What are the 6 common system involvements in sarcoidosis?

A
  1. Lungs
  2. Lymph nodes
  3. Joints
  4. Liver
  5. Skin
  6. Eyes
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7
Q

What type of granuloma does sarcoidosis have?

A

Non-caseating

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8
Q

What patients is sarcoidosis less common in?

A

Smokers

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9
Q

What are the two types of sarcoidosis?

A

Acute

Chronic

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10
Q

Give 5 clinical features of acute sarcoidosis?

A
Erythema nodosum
Bilateral hilar lymphadenopathy
Arthritis
Uveitis, parotitis
Fever
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11
Q

Give 5 clinical features of chronic sarcoidosis?

A
Lung infiltrates (alveolitis)
Skin infiltrations
Peripheral lymphadenopathy
Hypercalcaemia
Other organs: renal, myocardial, neurologicalm hepatitis, splenomegaly
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12
Q

Give four differential diagnosises of sarcoidosis?

A

TB (tuberculin test -ve)
Lymphoma
Carcinoma
Fungal infection

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13
Q

On a CXR of sarcoidosis what will you see?

A

Bilateral hilar lymphadenopathy

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14
Q

On a CT scan of lungs with sarcoidosis, what might you see?

A

Peripheral nodular infiltrate

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15
Q

What would you look for in a tissue biopsy of sarcoidosis?

A

Non-caseating granuloma

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16
Q

In pulmonary function of sarcoidosis, what causes the restrictive defect?

A

Lung infiltrates

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17
Q

With a blood test for sarcoidosis, what 3 things would suggest diagnosis?

A
  1. Angiotensin Converting Enzyme (ACE) levels as activity marker
  2. Raised calcium
  3. Increased inflammatroy markers
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18
Q

What is the treatment for acute sarcoidosis?

A

Self-limiting condition = no treatment, steroids if vital organ affected

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19
Q

What is the treatment for chronic sarcoidosis?

A

Oral steroids

Immunosuppression (e.g. azathioprine, methotrexate, anti-TNF therapy)

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20
Q

What clinical feature of the eyes can suggest sarcoidosis?

A

Iritis

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21
Q

What is another term for extrinsic allergic alveolitis?

A

Hypersensitivity pneumonitis

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22
Q

What type of reaction is extrinsic allergic alveolitis?

A

Type III hypersensitivity (immune complex deposition) reaction to antigen

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23
Q

Give three causes of extrinsic allergic alveolitis?

A
  1. Thermophilic actinomycetes (farmers lung, malt workers, mushroom workers)
  2. Avian antigens (bird fanciers lung)
  3. Drugs (gold, bleomycin, sulphasalazine)
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24
Q

What does this description describe: cough, breathless, fever, myalgia, symptoms occur several hours after acute exposure, signs (+/- pyrexia, crackles, no wheeze, hypoxia) and on CXR widespread pulmonary infiltrates?

A

Acute extrinsic allergic alveolitis

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25
Q

What is the treatment for acute extrinsic allergic alveolitis?

A

Oxygen
Steroid
Antigen avoidance

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26
Q

What causes chronic extrinsic allergic alveolitis?

A

Repeated low dose antigen exposure over time (years)

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27
Q

What condition does this describe: Progressive breathlessness and cough, signs (may be crackles, clubbing is unusual), CXR = pulmonary fibrosis - most commonly in the upper zones?

A

Chronic extrinsic allergic alveolitis

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28
Q

What will the pulmonary function tests be like in chronic extrinsic allergic alveolitis?

A
Restrictive defect
(Low FEV1 & FVC, high or normal ratio, low gas transfer - TLCO)
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29
Q

What three factors do you use to diagnose chronic extrinsic allergic alveolitis?

A

History of exposure

Precipitins (IgG antibodies to guily antigen, lung biopsy if in doubt)

30
Q

What treatment is there for chronic extrinsic allergic alveolitis?

A

Remove antigen exposure, oral steroids if breathless or low gas transfer

31
Q

What is formerly known as cryptogenic fibrosing alveolitis?

A

Idiopathic pulmonary fibrosis

32
Q

Is idiopathic pulmonary fibrosis an inflammatory disease?

A

No

N.B More common in smokers

33
Q

What are rheumatoid, SLE, systemic sclerosis, asbestos, (drugs - amiodarone, busulphan, bleomycin, penicillamine, nitrofurantoin, methotrexate all secondary causes of?

A

Pulmonary fibrosis

34
Q

What would you find on clinical presentation of idiopathic pulmonary fibrosis?

A

Progressive breathlessness (several years) and dry cough

35
Q

What would you find on examination of idiopathic pulmonary fibrosis?

A

Clubbing

Bilateral fine inspiratory crackles

36
Q

What would you find on investigation of idiopathic pulmonary fibrosis, including PFTs?

A

Restrictive defect on PFTs - reduced FEV1 and FVC with normal or raised FEV1/FVC ratio, reduced lung volumes and low gas transfer

37
Q

What would you see on CXR of idiopathic pulmonary fibrosis?

A

Bilateral infiltrates

38
Q

What might you see on a CT scan in idiopathic pulmonary fibrosis?

A

Reticulonodular fibrotic change, worse at the lung bases. The presence of “ground-glass” suggests reversible alveolitis; fibrosis is irreversible.

39
Q

Give 4 differential diagnosis of idiopathic pulmonary fibrosis?

A
  1. Mitral valve disease
  2. Left ventricular failure
  3. Sarcoidosis
  4. Extrinsic allergic alveolitis
40
Q

Describe the pathology of idiopathic pulmonary fibrosis?

A

Chronic inflammatory infiltrate (neutrophils and fibrosis in alveolar walls +/- intra-alveolar macrophages)

41
Q

What is coal workers pneumoconiosis?

A

Simple pneumoconiosis

42
Q

What is there an abnormality with in coal workers pneumoconiosis?

A

CXR (no impairment of lung function - often associated with chronic obstructive pulmonary disease)

43
Q

What does complicarted pneumoconiosis lead to?

A

Progressive massive fibrosis = restrictive pattern with breathlessness

44
Q

What is Caplan’s syndrome?

A

Rheumatoid pneumoconiosis (pulmonary nodules)

45
Q

What causes silicosis?

A

15-20 years exposure to quartz (e.g. mining, foundry workers, glass workers, boiler workers)

46
Q

What disease has egg-shell calcification of hilar nodes?

A

Silicosis

47
Q

What lung pattern is present in chronic silicosis?

A

Restrictive pattern

48
Q

What do mining, construction, shipbuilding, boilers and piping and automotive components like brake linings all have in common?

A

Asbestos exposure

49
Q

Name 4 features of pleural disease caused by asbestos?

A
  1. Benign pleural plaques - asymptomatic
  2. Acute asbestos pleuritis - fever, pain, bloody pleural effusion
  3. Pleural effusion and diffuse pleural thickening - restrictive impairment
  4. Maligannt mesothelioma - incurable pleural cancer.
50
Q

How does malignant mesothelioma present?

A

With chest pain and pleural effusion

51
Q

What does heavy, prolonged exposure of asbestos cause?

A

Asbestosis - pulmonary fibrosis

52
Q

Where can you find asbestos in a patient with asbestosis?

A

Asbestos bodies in sputum

Asbestos fibres in lung biopsy

53
Q

What is interstitial disease?

A

Any disease process affecting lung interstitium (i.e. alveoli, terminal bronchi)

54
Q

What is a thin elastin-rich connetive componenet containing capillary blood vessels?

A

Pulmonary interstitium

55
Q

What cells are present in pulmonary interstitium?

A

Alveolar lining cells

56
Q

What are the two stages in interstitial lung disease?

A
  1. Early stage is alveolitis (injury with inflammatory cell infiltration)
  2. Late stage characterised by fibrosis
57
Q

What are the two biopsies in interstitial lung disease?

A

Transbronchial biopsy - special forceps used at bronchoscopy

Thoracoscopic biopsy - more invasive but more reliable and generates far more tissue

58
Q

Name 5 chronic intersitial diseases?

A
  1. Fibrosing alveolitis
  2. Sarcoidosis
  3. Extrinsic allergic alveolitis (hypersensitivity pneumonitis)
  4. Pneumoconiosis
  5. Connective tissue disease
59
Q

What involves progressive interstitial fibrosis or unknown cause, variable associated inflammation and finger clubbing?

A

Fibrosing alveolitis

60
Q

What are three pathalogical features of fibrosing alveolitis?

A

Subpleural and basal fibrosis
Inflammatory component variable
Terminally lung structure replaced by dilated spaces surrounded by fibrous walls

61
Q

What condition does late honeycombing on histology appear in?

A

Fibrosing alveolitis

62
Q

Extrinsic allergic alveolitis can either be a chronic inflammatory disease or allergic origin - what are the three features of chronic inflammatory disease?

A

Small airways
Interstitium
Occasional granulomas

63
Q

What does thermophilic bacteria cause?

A

Farmer’s lung

64
Q

What do avian proteins cause?

A

Bird fanciers lung

65
Q

What does fungi cause?

A

Malt workers lung

66
Q

What is uveitis (occurs with sarcoidosis)?

A

Inflammation of iris

67
Q

Name 3 pulmonary involvements in connective tissue diseases?

A
  1. Interstitial fibrosis (milder than fibrosing alveolitis)
  2. Pleural effusions
  3. Rheumatoid nodules
68
Q

What is pneumoconiosis? Give examples?

A

Lung disease caused by mineral dust exposure. Examples include asbestosis, coal workers lung and silicosis

69
Q

What is asbestos?

A

A silicate

70
Q

What asbestos fibres are safe and which ones are dangerous?

A

Serpentine (curved) asbestos fibres are safe

Straight (amphibole) asbestos highly dangerous

71
Q

What four things can asbestos cause?

A
  1. Parietal pleural plaques
  2. Interstitial fibrosis (asbestosis)
  3. Bronchial carcinoma
  4. Mesothelioma