Interstitial and Occupational Lung Disease (DISEASE MECHANISMS) Flashcards

1
Q

What is interstitial disease?

A

Any disease that affects lung insterstitium, interferes with gas transfer and shows a restrictive lung pattern

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2
Q

What are the symptoms of ILD?

A

Breathlessness

Dry cough

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3
Q

What type of ILD would a viral infection cause?

A

Acute ILD

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4
Q

What can cause episodic ILD?

A

Environmental or systemic factors

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5
Q

What can cause chronic ILD?

A

Exposure to an external agent (drug, dust, etc.) or idiopathic

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6
Q

What is sarcoidosis?

A

Multi-system idiopathic non-caseating granulomatous type IV hypersensitivity disease

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7
Q

Which tissues does sarcoidosis commonly affect?

A
Lungs
Lymph nodes
Joints
Liver
Skin 
Eyes
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8
Q

Which tissues does sarcoidosis less commonly affect?

A

Kidneys
Brain
Nerves
Heart

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9
Q

What is the common presentation of acute sarcoidosis?

A
Uveitis
Erythema nosodum
BHL
Arthritis
Parotitis 
Fever
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10
Q

What is the common presentation of chronic sarcoidosis?

A

Lung infiltrates (Alveolitis)Skin infiltrations
Peripheral lymphadenopathy
Hypercalcaemia
Other organ involvement

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11
Q

What would be included in the differential diagnosis for sarcoidosis?

A

Tuberculosis
Lymphoma
Carcinoma
Fungal infection

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12
Q

How can you test for sarcoidosis?

A
CXR
CT scan thorax
Tissue biopsy
Pulmonary function tests
Blood tests
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13
Q

What would a CR of a sarcoidosis patient show?

A

BHL

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14
Q

What would a CT scan thorax of a sarcoidosis patient show?

A

Peripheral nodular infiltrate

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15
Q

What would a tissue biopsy of a sarcoidosis patient show?

A

Non-Caseating granuloma (diagnostic)

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16
Q

What type of lung pattern do sarcoidosis patients have?

A

Restrictive

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17
Q

What would be the expected blood test results be a for a sarcoidosis patient?

A

Increase ACE
Increased Ca
Increased Immunoglobulins

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18
Q

How is acute sarcoidosis treated?

A

Self-limiting
Bed Rest
NSAIDs (e.g. aspirin)

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19
Q

How is chronic sarcoidosis treated?

A

Oral steroid if vital organs affected

Immunosuppressants in severe illness

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20
Q

What type of follow-up should sarcoidosis patients receive?

A

Monitoring of CXR and pulmonary function for years to check for possible relapse

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21
Q

What are the indications for corticosteroids in sarcoidosis?

A

Parenchymal lung disease
Uveitis
Hypercalcaemia
Neurological or cardiac involvement

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22
Q

What is extrinsic allergic alveolitis?

A

Type III hypersensitivity reaction to an antigen

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23
Q

What are the causes of EAA?

A

Farmer’s and Mushroom’s lung (Micropolyspora faeni, Thermoactinomyces vulgaris)
Malt worker’s lung (aspergillus cluvatus)
Bagassosis or sugar worker’s lung (Thermoactinomyces sacchari)
Drugs (Bleomycin, gold, sulphazalanine)

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24
Q

What are the clinical features of acute EAA?

A
Cough
Breathless
Myalgia
Fever/Pyrexia
Crackles
Hypoxia
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25
Q

How is acute EAA treated?

A
Remove allergen
Give O2 (35-60%) 
Then oral prednisolon (40mg/24h PO) followed by reducing dose
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26
Q

What tests can be undertaken for acute EAA?

A

Blood - FBC, increased ESR, ABGs, positive serum precipitins (indicate exposure only)

CXR - upper zone mottling/consolidation, hilar lymphadenopathy

PFTs - restrictive pattern

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27
Q

What would cause chronic EAA?

A

Recurrent low antigen exposure

28
Q

What are the signs and symptoms of chronic EAA?

A
Progressive Dyspnoea
Cough
Crackles
Weight loss
Exertion dyspnoea
Type I respiratory failure 
Cor pulmonale
CXR - fibrosis (mainly upper zones) 
PFTs - restrictive
29
Q

How can a diagnosis of chronic EAA be made?

A

History of exposure
Positive serum precipitins
Lung biopsy if doubt
BAL (Bronchial alveolar lavage) shows increased lymphocytes & mast cells

30
Q

How is chronic EAA treated?

A

Avoid exposure to allergen or wear facemark for positive pressure helmet
Long-term steroids often achieve CXR + physiological improvement

31
Q

What is idiopathic pulmonary fibrosis?

A
Unknown origin
Imbalance of fibrotic repair system
?related to gastric reflux 
Not inflammatory
More common in smokers
32
Q

What are secondary causes of pulmonary fibrosis?

A
Rheumatoid
SLE
Systemic sclerosis
Asbestos
Drugs (amiodarone, bleomycin, penicillamine, nitrofurantoin, methotrexate)
33
Q

What is the clinical presentation of IPF?

A

Progressive dyspnoea

Dry cough

34
Q

What is found O/E for IPF?

A

Clubbing

Bilateral fine inspiratory crackles

35
Q

What is found Ix for IPF?

A

Restrictive pattern on PFTs

CXR - bilateral infiltrates, reduced lung volume, bilateral zone reticulonodular shadows, honeycomb (if advanced)

CT - reticulonodular fibrosis shadowing, worse at lung bases and periphery / Traction bronchiectasis, honey-combing / cystic changes

Lung biopsy (only if CT not diagnostic

Bloods: ABGs (low PaO2, high PaCO2 if severe) / CRP increased, increased immunoglobulins / ANA (30% TVC) / Rheumatoid factor (10% +ve)

36
Q

What would have to be excluded from the differential to confirm IPF?

A

Occupational disease (asbestosis, silicosis)
Connective tissue disease (Rha, scleroderma, Sjorgens disease, SLE)
LVT
Sarcoidosis
EAA

37
Q

How is the diagnosis of IPF made?

A

Combination of history, examination and HRCT scan (radiology)

38
Q

What is the pathology of IPF?

A

Usual interstitial pneumonia pattern: heterogeneous fibres fibrosis in alveolar walls with fibroblastic foci and destruction of architecture causing honeycombing.
Inflammation is minimal

39
Q

How is IPF treated?

A

Antifibrotic drugs (slow progression, no reversal) are in clinical trials
No steroids or anti-inflammatories (unless doubt diagnosis)
lung transplant
Best supportive care: O2 if hypoxic, pulmonary rehabilitation, opiates, palliative care input

40
Q

What is the prognosis of IPF patients?

A

50% 5yr survival rate

Range 1-20 years

41
Q

What does coal worker’s simple pneumoconiosis show on CXR?

A

Abnormality

42
Q

What is the effect of coal worker’s simple pneumoconiosis on lung function?

A

Often associated with COPD

No impairment of lung function

43
Q

What does coal worker’s complicated pneumoconiosis cause?

A

Progressive massive fibrosis (PMF)
Dyspnoea
Eventually cor pulmonale

44
Q

What kind of lung pattern do patient’s with coal worker’s complicated pneumoconiosis have?

A

Restrictive

45
Q

What causes chronic bronchitis?

A

Coal dust + smoking

46
Q

What is Caplan’s syndrome?

A

Association between rheumatoid arthritis, pneumoconiosis, and pulmonary rheumatoid nodules

47
Q

What causes silicosis?

A

Inhalation of silica particles (exposure to quartz - usually over long time

48
Q

What occupations is silicosis associated with?

A

Mining
Foundry workers
Glass workers
Boiler workers

49
Q

How does simple pneumoconiosis silicosis present?

A

Few symptoms

CXR - egg-shell calcification of hilar nodes

50
Q

How does chronic pneumoconiosis silicosis present?

A

Progressive dyspnoea
Increased incidence of TB

CXR - diffuse military or nodular patterning upper and mid zones + egg-shell calcification of hilar nodes

Spirometry – restrictive pattern

51
Q

What causes baritosis?

A

Exposure to barium

52
Q

What are asbestos related pleural diseases?

A

Benign pleural plaques
Acute asbestos pleuritis
Pleural effusion and diffuse pleural thickening
Malignant mesothelioma

53
Q

What are non-pleural asbestos related diseases?

A

Asbestosis

Bronchial carcinoma

54
Q

What symptoms do benign pleural plaques cause?

A

None

55
Q

How does acute asbestos pleuritis present?

A

Fever
Pain
Bloody pleural effusion

56
Q

How does pleural effusion and diffuse pleural thickening due to asbestosis present in terms of spirometry?

A

Restrictive impairment

57
Q

What is malignant mesothelioma?

A

Incurable pleural cancer

58
Q

How does malignant mesothelioma present?

A

Chest pain

Pleural effusion

59
Q

What is the average prognosis for malignant mesotheliomas

A

2 years

60
Q

What colour is chrysotile asbestos?

A

white

61
Q

What colour is amosite asbestos?

A

brown

62
Q

What colour is crocidolite asbestos?

A

blue

63
Q

In which order do the different colours of asbestos rank for increasing fibrogenicity?

A

White - brown - blue

64
Q

What are the clinical features of asbestosis?

A
Progressive dyspnoea 
Clubbing
Fine end-inspiratory crackles 
Pleural plaques 
Increased risk of bronchial adenocarcinoma and mesothelioma
65
Q

The fact that smokers that have been exposed to asbestos have a higher risk for lung cancer than the sum of asbestos and smoker risk is called the ______?

A

Synergistic effect

66
Q

Which occupations are related to asbestos exposure?

A

Mining
Construction
Ship-building
Boilers and piping