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Respiratory > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

What does restrictive lung disease mean?

A

Diseases of the lung itself (alveoli & interstitium) that restrict lung expansion due to stiffness

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2
Q

What are key tests in the diagnosis of RLD

A

FVC/FEV1 ratio (>70) and DLCO (<80%)

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3
Q

What does DLCO measure?

A

Gas diffusion across alveolar-arterial barrier

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4
Q

Give some structural, neural and interstitial causes of RLD…

A
  1. kyphosis-scoliosis, obesity, diaphragmatic palsy
  2. motor-neurone disease
  3. mesothelioma, pleural ef
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5
Q

What is a common complication of RLD

A

Hypoventilation

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6
Q

True/False:

Interstitial Lung Disease is the most common type of RLD?

A

True

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7
Q

What diseases are encompassed under ‘Acute ILD’ category?

A

Respiratory Distress Syndrome, Alveolitis

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8
Q

What diseases are encompassed under ‘Chronic ILD’?

A

Idiopathic Pulmonary Fib, Hypersensitivity Pneumonitis, Coal-workers Pneumoconiosis, Asbestosis, Silicosis (among others)

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9
Q

What characterises Chronic ILDs?

A

Fibrosis

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10
Q

What is the epidemiology of Idiopathic Pulmonary Fibrosis (IPF)

A

males more at risk, over 50 years of age

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11
Q

What is IPF?

A

repeated injury of alveolar epithelium resulting in fibrosis of tissue.

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12
Q

What is the pathophysiology of IPF?

A

Repeated injury to alveolar epi > uncontrolled healing leading to hyper production of fibroblasts > loss of structural integrity of lung parenchyma = stiffening

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13
Q

Aetiology of IPF?

A

unknown. may be due to inhalation insults, viruses, drugs, GORD*, genetic

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14
Q

What specific drug may be responsible for IPF?

A

methotrexate

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15
Q

What are the presenting symptoms of IPF?

A
  • progressive dyspnoea & dry cough, wt loss

- clubbing, bi-basal inspiratory crackles, cyanosis

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16
Q

Ix for IPF…

A

PFTs, blood tests, CXR, HRCT, bronchoalveolar lavage

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17
Q

What could be seen on CXR and HRCT if IPF is present?

A

CXR: bilateral infiltrates, many lines and dots
HRCT: honeycombing

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18
Q

What bloods would be done for IPF?

A

RF, ANA (to exclude rheumatic disease) , ABGs

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19
Q

What are ‘many lines and dots’ expressed as on a CXR?

A

Reticulonodular pattern

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20
Q

Tx for IPF…

A

transplant, anti-fibrotic therapy, supportive (O2, pulmonary rehab)

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21
Q

What are complications of IPF?

A

cor pulmonale (RVHF), resp failure (CO2 retention)

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22
Q

What is the prognosis for IPF?

A

2-4 years so very poor :(

life expectancy shorter after acute exacerbations

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23
Q

What is the other name for Hypersensitivity Pneumonitis?

A

Extrinsic Allergic Alveolitis

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24
Q

What are the classes of HP?

A

Acute, subacute, chronic

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25
Q

What type of hypersensitivity reaction is subacute HP?

A

Type 3

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26
Q

What is HP?

A

Allergic reaction affecting small airways and alveoli in response to an inhaled antigen

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27
Q

What is the pathophysiology of HP?

A

Chronic infiltrate which triggers an immunological and inflammatory response which activates macrophages resulting in progressive development of pulmonary fibrosis

28
Q

What are the triggers for HP…

A

Bacterial: farmer’s lung, humidifier’s lung
Protein: Bird-fancier’s lung
Fungi: Malt-worker’s Lung, mushroom, cheese washer, winemaker

29
Q

What characteristic does bacteria responsible for HP usually possess?

A

it is usually Thermophilic (thrives in high ºC)

30
Q

What are the general presenting symptoms for HP?

A

dyspnoea & dry cough, inspiratory wheeze, bilateral crackles

31
Q

Discuss acuteHP in terms of symptoms and resolution…

A

fever, rigors, malaise, cough & dyspnoea, hypoxia
comes on: 4-6hrs after exposure
resolves: 24-48hrs after exposure

32
Q

Discuss subacute HP in terms of symptoms and resolution…

A

wt loss *
comes on: low level/ intermittent exposure
resolves: within weeks

33
Q

Discuss chronic HP in terms of symptoms and resolution…

A

wt loss, dyspnoea & cough, cor pulmonale
comes on: repeated exposure
resolves: never

34
Q

Ix for HP…

A

CXR, HRCT, PFTs, lung biopsy

35
Q

True/False:

CXR for acute HP shows apex pulmonary fibrosis

A

False

CXR for acute HP shows infiltrates, for chronic HP shows apex PFib

36
Q

Tx for HP…

A

acute: O2, oral steroids, anti-fib therapy
chronic: oral steroids, avoid antigen

37
Q

True/False:

Prognosis for HP is good

38
Q

What are the 3 main conditions encompassed by occupational lung disease?

A

Silicosis, Coal-workers pneumoconiosis, Asbestosis

39
Q

Is Pneumoconiosis common?

40
Q

Is silicosis common?

41
Q

What are the 4 different types of pneumoconiosis?

A

simple pneumoconiosis, complicated pneumoconiosis (progressive massive fibrosis), co-existing bronchitis pneumoconiosis, caplan’s syndrome (rheumatoid pneumoconiosis)

42
Q

True/False:

Hypersensitivity types 1,2,3 are present in simple pneumoconiosis?

43
Q

What are the 2 classes of silicosis and describe them?

A

simple (asymptomatic with CXR abnormalities), chronic (restrictive pattern & p fib occurs)

44
Q

How can asbestos present in the pleura? (5 points)

A

Asbestos bodies, pleural plaques, defuse pleural thickening, pleural effusion/ acute asbestos pleuritis (bloody pleural ef), mesothelioma

45
Q

How can asbestos present in other ways other than pleural infiltration?

A

Asbestosis (interstitial fibrosis), asbestos related bronchial carcinoma

46
Q

What is CW pneumoconiosis?

A

small dust particles that are retained in the small airways and alveoli of lungs

47
Q

What is silicosis?

A

inhalation of silica

48
Q

What is asbestosis?

A

fibrosis of lungs due to asbestos exposure

49
Q

What is mesothelioma?

A

tumour of lining of the lung- mix of sarcoma & carcinoma cells

50
Q

What is pathophysiology of occupational lung diseases (OLD)…

A
  • CWP: dust in lungs causes surrounding tissue to react and initiate an inflammatory response= fibrosis, emphysema & airway damage
  • Silicosis: silica is toxic to macrophages initiating rapid fibrogenesis
  • Asbestosis: inflammatory reaction to asbestos bodies resulting in diffuse interstitial fibrosis
51
Q

Triggers of OLD…

A

CWP: coal dust
Silicosis: long term exposure to stone (e.g. sandblasting)
Asbestosis: long term exposure to asbestos fibres e.g. building site

52
Q

True/False:

Bent asbestos fibres are more dangerous that straight asbestos fibres?

53
Q

Presenting symptoms of CWP…

A

dyspnoea & dry cough, black sputum

54
Q

Presenting symptoms of silicosis…

A

dyspnoea & dry cough

55
Q

Presenting symptoms of asbestosis…

A

progressive dyspnoea & cough, clubbing, basal inspiratory crackles

56
Q

Presenting symptoms of mesothelioma…

A

effusion, chest wall pain, dyspnoea, wt loss,

57
Q

OLD ix…

A

PFTs, CXR, HRCT

58
Q

CWP CXR findings..

A

many round opacities in upper zones

59
Q

silicosis CXR findings..

A

thin streaks of calcification around hilar lymph nodes, opacities in upper lung

60
Q

Asbestosis CXR findings…

A

honeycombing and streaky shadowing

61
Q

Ix for mesothelioma…

A

thorascopic pleural biopsy, thoracentesis*, CXR

62
Q

Tx for OLD..

A

CWP: avoid exposure, treat bronchitis
Silicosis: avoid exposure
Asbestosis: corticosteroids

63
Q

True/False:

A complication of asbestosis is inc risk of cancer?

64
Q

Tx for mesothelioma…

A

surgery, chemo, palliative, pleurodesis (obliteration of pleural cavity)

65
Q

Prognosis for OLD?

A

All involve pulmonary fibrosis so bad prognosis

66
Q

Prognosis for mesothelioma?

67
Q

Is compensation available for OLD and for HP?

Respiratory (19 decks)

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