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Study notes - Respiratory > *Interstitial and Occupational Lung Diseases 1 (lecture 1, 2 and 3) > Flashcards

*Interstitial and Occupational Lung Diseases 1 (lecture 1, 2 and 3) Flashcards

1
Q

What are interstitial diseases of the lung?

A

Any disease process affecting the lung interstitium/ parenchyma in a diffuse manner i.e. alveoli and terminal bronchi

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2
Q

So interstitial diseases have a restrictive or obstructive lung pattern?

A

Restrictive

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3
Q

What are the characteristic features of interstitial lung disease in terms of the lung tissue?

A

Chronic inflammation

+/- progressive interstitial fibrosis

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4
Q

2 main symptoms of an interstitial lung disease?

A

Breathlessness

Dry cough

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5
Q

What are the 3 main categories, in terms of time, of interstitial lung disease?

A

Acute
Episodic
Chronic (part of systemic disease, exposure to agent, idiopathic)

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6
Q

What are the 4 main types of interstitial lung diseases?

A

ILD of known cause or association
Idiopathic interstitial pneumonia
Granulomatous ILDs
Other forms of ILD

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7
Q

How is idiopathic interstitial pneumonia categorised?

A

Either idiopathic pulmonary fibrosis

Idiopathic Interstitial pneumonia that isn’t IPF

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8
Q

What are the 6 causes of idiopathic interstitial pneumonia that are not idiopathic pulmonary fibrosis?

A

Desquamative interstitial pneumonia
Respiratory bronchiolitis interstitial lung disease
Acute interstitial pneumonia
Cryptogenic organising pneumonia
Lymphocytic interstitial pneumonia
Non-specific interstitial pneumonia (NSIP)

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9
Q

What are the 2 most important granulomatous ILDs to remember?

A

Sarcoidosis

Extrinsic allergic alveolitis

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10
Q

What is sarcoidosis?

A

A granulomatous disease of unknown cause

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11
Q

What type of hypersensitivity reaction is sarcoidosis?

A

Type 4

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12
Q

What systems are commonly affected by sarcoidosis? (6)

What systems are less commonly affected by sarcoidosis? (4)

A

Commonly:
Lungs, lymph nodes, joints, liver, skin, eyes
Less commonly:
Kidneys, brain, nerves, heart

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13
Q

Are the granulomas in sarcoidosis caseating or non-caveating?

A

non-caseating

TB = caseating

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14
Q

Is sarcoidosis more or less common in smokers?

A

Less

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15
Q

What are the 2 types of sarcoidosis?

A

Acute

Chronic

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16
Q

What are the symptoms of acute sarcoidosis?

A 
Erythema nodosum
Bilateral hilar lymphadenopathy
Arthritis
Uveitis, parotitis
Fever
(patient will feel generally unwell)
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17
Q

Symptoms of chronic sarcoidosis?

A 
Lung infiltrates (alveolitis)
Skin infiltrations
Peripheral lymphadenopathy
Hypercalcaemia
Other organs = renal, myocardial neurological, hepatitis, splenomegaly
(symptoms come on more insidiously)
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18
Q

Investigations for sarcoidosis? (5)

A

Chest x-ray (BHL)
CT scan of lungs (peripheral nodular infiltrate)
Tissue biopsy (e.g. transbronchial, skin, lymph node) for non-caeseating granuloma
Pulmonary function (restrictive lung pattern)
Blood tests (ACEI as activity marker - not diagnostic test, raised calcium, raised inflammatory markers)

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19
Q

Treatment of acute sarcoidosis?

A

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

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20
Q

Treatment of chronic sarcoidosis?

A

Ora steroids usually needed
Immunosupression e.g. aathioprine, methotrexate, anti-TNF therapy
(monitor chest x-ray and pulmonary function for several years as often relapses)

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21
Q

What is another name for extrinsic allergic alveolitis?

A

Hypersensitivity pneumonitis

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22
Q

What type of hypersensitivity is extrinsic allergic alveolitis?

A

Type III hypersensitivty (predominantly - there is also a type 4 reaction)

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23
Q

What happens in extrinsic allergic alveolitis?

A

In sensitised individuals, inhalation of allergens (fungal spores, thermophilic bacteria or avian proteins) causes a hypersensitivity reaction
In the acute phase, alveoli are infiltrated with acute inflammatory cells
With chronic exposure, granulomas form and obliterative bronchiolitis occurs

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24
Q

Types of extrinsic allergic alveolitis caused by exposure to protein in avian droplets?

A

Bird fanciers lung

Pigeon fanciers lung

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25
Q

Type of extrinsic allergic alveolitis due to exposure to aspergillus clavatus?

A

Malt workers lung

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26
Q

Type of extrinsic allergic alveolitis due to exposure to micropolyspora faeni usually seen in farmers caused by mouldy hay?

A

Farmers lung

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27
Q

Type of extrinsic allergic alveolitis typically seen in people who work with mushrooms due to exposure to thermoactinomyces vulgaris?

A

Mushroom workers lung

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28
Q

Drugs that can cause extrinsic allergic alveolitis? (3)

A

Gold
Bleomycin
Sulphasalazine (rheumatoid arthritis)
(no cause identified in approx 30% of cases of extrinsic allergic alveolitis)

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29
Q

2 types of extrinsic allergic alveolitis?

A

Acute

Chronic

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30
Q

Symptoms of acute extrinsic allergic alveolitis?

Onset of symptoms?

A 
Cough
Breathlessness
Fever
Myalgia
Classical several hours after acute exposure
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31
Q

Signs of acute extrinsic allergic alveolus?

A

+/- pyrexia
Crackles (no wheeze)
Hypoxia

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32
Q

Appearance of a chest x-ray in a patient with acute extrinsic allergic alveolitis?

A

Widespread pulmonary infiltrates

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33
Q

Treatment of extrinsic allergic alveolitis?

A

Oxygen
Steroid
Antigen avoidance

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34
Q

What causes chronic extrinsic allergic alveolitis?

A

Repeated low dose antigen exposure over time (years) leading to progressive breathlessness and a cough

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35
Q

Signs of chronic extrinsic allergic alveolitis?

A

Crackles - clubbing is unusual

36
Q

Appearance of chronic extrinsic allergic alveolitis on a chest x-ray?

A

Pulmonary fibrosis - most commonly in the upper zones

37
Q

Diagnosis of chronic extrinsic allergic alveolitis?

A

History of exposure, preciptins (IgG antibodies to guilty antigen), lung biopsy if in doubt

38
Q

Treatment of chronic extrinsic allergic alveolitis?

A

Remove antigen exposure

Oral steroids if breathless or low gas transfer

39
Q

Causes of fibrosis in the upper zones?

A 
TB
Extrinsic allergic alveolitis
Ankylosing spondylitis
Radiotherapy
Sarcoidosis
40
Q

Causes of fibrosis in the middle zones?

A

Progressive massive fibrosis

41
Q

Causes of fibrosis in the lower zone?

A

Idiopathic pulmonary fibrosis

Asbestosis

42
Q

Former name of idiopathic pulmonary fibrosis? (2)

A

Cryptogenic fibrosing alveolitis

Also known as usual interstitial pneumonia

43
Q

What is the most common interstitial lung disease?

A

IPF

44
Q

What is idiopathic pulmonary fibrosis?

A

Inflammatory cell infiltrate and pulmonary fibrosis of unknown cause (not predominantly an inflammatory condition and therefore doesn’t respond well to steroids)

45
Q

What is thought to be the cause of IPD?

A

imbalance of fibrotic repair mechanism - the lung is trying to repair itself by laying down scar tissue when there is nothing to be repaired

46
Q

Is IPF more or less common in smokers?

A

More

47
Q

Secondary causes of pulmonary fibrosis that my be ruled out before diagnosing idiopathic pulmonary fibrosis?

A

Rheaumatoid, lupus, systemic sclerosis, asbestos, drugs

48
Q

What drugs can cause pulmonary fibrosis?

A 
Amiodarone - anti-arrhythmic
Busulphan - cancer drug
Bleomycin
Penicillamine - wilsons disease
Nitrofuratoin -antibiotic for UTIs
Methotrexate - cancer, etc.
49
Q

Symptoms and signs of idiopathic pulmonary fibrosis?

A 
Progressive breathlessness (several years), dry cough
Clubbin, bilateral fine inspiratory crackles
50
Q

Investigations for IPF? (4)

A

PFTs (restrictive pattern)
CxR (bilateral infiltrates)
CT scan (reticulonodular fibrotic change)
Lung biopsy - not needed if CT is diagnostic

51
Q

What is a reticular interstitial pattern?

A

Complex network of opacities that normally affect the lung diffusely

52
Q

Reticulonodular interstitial pattern?

A

produced by either, overlap of reticular shadows, or by the presence of reticular shadowing and pulmonary nodules.

53
Q

3 types of reticular interstitial pattern?

A

fine “ground-glass” (1-2 mm): seen in processes that thicken the pulmonary interstitium to produce a fine network of lines, e.g. interstitial pulmonary oedema
medium “honeycombing” (3-10 mm): commonly seen in pulmonary fibrosis with involvement of the parenchymal and peripheal interstitium
coarse (> 10 mm): cystic spaces caused by parenchymal descruction, e.g. usual interstitial pneumonia, pulmonary sarcoidosis, pulmonary Langerhans cell histiocytosis

54
Q

What does the presence of a ground glass fibrotic change indicate?

A

Reversible alveolitis - fibrosis is irreversible

55
Q

What would the pathological appearance of idiopathic pulmonary fibrosis be?

A

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

56
Q

Treatment of IPF?

A

Supportive therapy e.g. O2, opiates, palliative care
N-acetyl cistein may have anti-fibrotic effects via its anti-oxidant properties
Pirfenidone is a new antifibrtoic drug that slows down the progression of the disease but very expensive
If the patient is young = lung transplant

57
Q

What is pneumoconiosis?

A

a disease of the lungs due to inhalation of dust, characterized by inflammation, coughing, and fibrosis.

58
Q

What is coal workers pneumoconiosis?

A

A common dust disease resulting from inhalation of coal dust particles over 15- 20 years - ingested by macrophages which die, releasing their enzymes and causing fibrosis

59
Q

What is simple pneumoconiosis? (coal workers pneumoconiosis)

A

A chest x-ray abnormality (no impairment of the lung function) - often with coexisting chronic bronchitis

60
Q

What is progressive massive fibrosis?

A

Due to progression of CWP - restrictive pattern with breathlessness

61
Q

Caplan’s syndrome?

A

The association between rheumatoid arthritis, pneumoconiosis and pulmonary rheumatoid nodules

62
Q

What is silicosis?

A

Occupational lung disease caused by inhalation of silica particles (can be wither simple or chronic)

63
Q

Simple silicosis?

A

Chest x-ray abnormality only (egg shell calcification of hillier nodes)

64
Q

Chronic silicosis?

A

Restrictive pattern, pulmonary firbossi

65
Q

What 3 categories of disease can asbestos cause?

A

Pleural disease
Pulmonary fibrosis
Bronchial carcinoma (asbestos multiplies risk in smokers)

66
Q

What 4 diseases of the pleura can asbestos cause?

A

Benign pleural plaques - asymtpomatic
Acute asbestos pleuritic - fever, pain, bloody pleural effusion
Pleural effusion and diffuse pleural thickening - restrictive impairment
Malignant mesothelioma

67
Q

What is the name for pulmonary fibrosis due to asbestos exposure?
Diagnosis?

A

Asbestosis - heavy prolonged exposure hisotry, diffuse pulmonary fibrosis and restrictive defect, asbestos bodies in sputum, asbestos fibres in lung biopsy

68
Q

What is the pulmonary interstitium made up of? (2)

A

Alveolar lining cells (types 1 and 2)

Thin elastin-rich connective component containing capillary blood vessels

69
Q

In terms of pathology, what are the 2 stages of interstitial lung disease?

A

Early stage = alveolitis (injury with inflammatory cell infiltration)
Late stage = fibrosis

70
Q

What type of heart failure is associated with late stage lung disease?

A

Right sided heart failure

71
Q

What are the 2 possible types of biopsy that can be take for interstitial lung disease?

A

Transbronchial biopsy

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

72
Q

What are the main types of chronic interstitial diseases?

A 
Pulmonary fibrosis (fibrosing alveolitis)
Sarcoidosis
Extrinsic allergic alveolitis
Pneumoconiosis
Connective tissue diseases
73
Q

Where about does the fibrosis caused by idiopathic pulmonary fibrosis occur?

A

Subpleural

Basal fibrosis

74
Q

What is honeycomb lung?

A

the radiological appearance seen with widespread fibrosis and is defined by the presence of small cystic spaces with irregularly thickened walls composed of fibrous tissue = poor prognosis (dilated spaces surrounded by fibrous walls)

75
Q

Types of asbestos fibres from most to least fibrogenic?

A

Crocidolite (blue) = most (straight - amphibole)
Amosite (brown) = intermediate
Chrysotile (white) = least (curved - serpentine)

76
Q

What are the 2 basic types of occupational lung disease?

A

Extrinsic allergic alveolitis

pneumoconiosis

77
Q

What is the type of EAA caused by exposure to loudly sugar cane fibre - thermoactinomyces saccharin?

A

Bagossosis/ sugar handlers lung

78
Q

What is the type of EAA caused by exposure to contaminated forced-air systems/ heated water reservoirs - s rectivigula/ T vulgaris?

A

Humidifier/ air-conditioner lung

79
Q

What is the type of EAA caused by exposure to cheese mould? (penicillin casei)

A

Cheese worker’s lung

80
Q

How lung after exposure to asbestos does mesothelioma develop?

A

Over 20 years (usually 30 - 40)

81
Q

What 2 factors added together have a synergistic effect on increasing chance of developing lung cancer?

A

Smoking

Asbestos exposure

82
Q

What type of conditions cause mould to grow in houses?

A

Damp and cold conditions

83
Q

Environmental sources of lung disease? (not occupational)

A 
Asbestos
Mould
Cooking smoke
Passive smoking
Air/ traffic pollution
Nanoparticles
84
Q

What are dioxins?

A

A sub-set of persistent organic compounds that are associated with reproductive and immunological problems - bioaccumulates in people and animal fat (at least 90% come from out diet)

85
Q

What is a notifiable disease?

A

A notifiable disease is any disease that is required by law to be reported to government authorities e.g. legionella

Study notes - Respiratory (29 decks)

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