Interstitial and Occupational Lung Diseases 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

BreathlessnessDry cough

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5
Q

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

A

AcuteEpisodicChronic (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 associationIdiopathic interstitial pneumoniaGranulomatous ILDsOther forms of ILD

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7
Q

How is idiopathic interstitial pneumonia categorised?

A

Either idiopathic pulmonary fibrosisIdiopathic 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 pneumoniaRespiratory bronchiolitis interstitial lung diseaseAcute interstitial pneumoniaCryptogenic organising pneumoniaLymphocytic interstitial pneumoniaNon-specific interstitial pneumonia (NSIP)

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9
Q

What are the 2 most important granulomatous ILDs to remember?

A

SarcoidosisExtrinsic 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, eyesLess 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

AcuteChronic

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16
Q

What are the symptoms of acute sarcoidosis?

A

Erythema nodosumBilateral hilar lymphadenopathyArthritisUveitis, parotitisFever(patient will feel generally unwell)

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17
Q

Symptoms of chronic sarcoidosis?

A

Lung infiltrates (alveolitis)Skin infiltrationsPeripheral lymphadenopathyHypercalcaemiaOther 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 granulomaPulmonary 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 neededImmunosupression 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 reactionIn the acute phase, alveoli are infiltrated with acute inflammatory cellsWith 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 lungPigeon 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

GoldBleomycinSulphasalazine (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

AcuteChronic

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30
Q

Symptoms of acute extrinsic allergic alveolitis?Onset of symptoms?

A

CoughBreathlessnessFeverMyalgiaClassical several hours after acute exposure

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31
Q

Signs of acute extrinsic allergic alveolus?

A

+/- pyrexiaCrackles (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

OxygenSteroidAntigen 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 exposureOral steroids if breathless or low gas transfer

39
Q

Causes of fibrosis in the upper zones?

A

TBExtrinsic allergic alveolitisAnkylosing spondylitisRadiotherapySarcoidosis

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 fibrosisAsbestosis

42
Q

Former name of idiopathic pulmonary fibrosis? (2)

A

Cryptogenic fibrosing alveolitisAlso 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-arrhythmicBusulphan - cancer drugBleomycinPenicillamine - wilsons diseaseNitrofuratoin -antibiotic for UTIsMethotrexate - cancer, etc.

49
Q

Symptoms and signs of idiopathic pulmonary fibrosis?

A

Progressive breathlessness (several years), dry coughClubbin, 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 oedemamedium “honeycombing” (3-10 mm): commonly seen in pulmonary fibrosis with involvement of the parenchymal and peripheal interstitiumcoarse (> 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 careN-acetyl cistein may have anti-fibrotic effects via its anti-oxidant propertiesPirfenidone is a new antifibrtoic drug that slows down the progression of the disease but very expensiveIf 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 diseasePulmonary fibrosisBronchial carcinoma (asbestos multiplies risk in smokers)

66
Q

What 4 diseases of the pleura can asbestos cause?

A

Benign pleural plaques - asymtpomaticAcute asbestos pleuritic - fever, pain, bloody pleural effusionPleural effusion and diffuse pleural thickening - restrictive impairmentMalignant 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 biopsyThoracoscopic 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)SarcoidosisExtrinsic allergic alveolitisPneumoconiosisConnective tissue diseases

73
Q

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

A

SubpleuralBasal 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) = intermediateChrysotile (white) = least (curved - serpentine)

76
Q

What are the 2 basic types of occupational lung disease?

A

Extrinsic allergic alveolitispneumoconiosis

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

SmokingAsbestos 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

AsbestosMouldCooking smokePassive smokingAir/ traffic pollutionNanoparticles

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