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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
What type of hypersensitivity reaction is subacute HP?
Type 3
26
What is HP?
Allergic reaction affecting small airways and alveoli in response to an inhaled antigen
27
What is the pathophysiology of HP?
Chronic infiltrate which triggers an immunological and inflammatory response which activates macrophages resulting in progressive development of pulmonary fibrosis
28
What are the triggers for HP...
Bacterial: farmer's lung, humidifier's lung Protein: Bird-fancier's lung Fungi: Malt-worker's Lung, mushroom, cheese washer, winemaker
29
What characteristic does bacteria responsible for HP usually possess?
it is usually Thermophilic (thrives in high ºC)
30
What are the general presenting symptoms for HP?
dyspnoea & dry cough, inspiratory wheeze, bilateral crackles
31
Discuss acuteHP in terms of symptoms and resolution...
fever, rigors, malaise, cough & dyspnoea, hypoxia comes on: 4-6hrs after exposure resolves: 24-48hrs after exposure
32
Discuss subacute HP in terms of symptoms and resolution...
wt loss * comes on: low level/ intermittent exposure resolves: within weeks
33
Discuss chronic HP in terms of symptoms and resolution...
wt loss, dyspnoea & cough, cor pulmonale comes on: repeated exposure resolves: never
34
Ix for HP...
CXR, HRCT, PFTs, lung biopsy
35
True/False: | CXR for acute HP shows apex pulmonary fibrosis
False | CXR for acute HP shows infiltrates, for chronic HP shows apex PFib
36
Tx for HP...
acute: O2, oral steroids, anti-fib therapy chronic: oral steroids, avoid antigen
37
True/False: | Prognosis for HP is good
True
38
What are the 3 main conditions encompassed by occupational lung disease?
Silicosis, Coal-workers pneumoconiosis, Asbestosis
39
Is Pneumoconiosis common?
yes
40
Is silicosis common?
no
41
What are the 4 different types of pneumoconiosis?
simple pneumoconiosis, complicated pneumoconiosis (progressive massive fibrosis), co-existing bronchitis pneumoconiosis, caplan's syndrome (rheumatoid pneumoconiosis)
42
True/False: | Hypersensitivity types 1,2,3 are present in simple pneumoconiosis?
True
43
What are the 2 classes of silicosis and describe them?
simple (asymptomatic with CXR abnormalities), chronic (restrictive pattern & p fib occurs)
44
How can asbestos present in the pleura? (5 points)
Asbestos bodies, pleural plaques, defuse pleural thickening, pleural effusion/ acute asbestos pleuritis (bloody pleural ef), mesothelioma
45
How can asbestos present in other ways other than pleural infiltration?
Asbestosis (interstitial fibrosis), asbestos related bronchial carcinoma
46
What is CW pneumoconiosis?
small dust particles that are retained in the small airways and alveoli of lungs
47
What is silicosis?
inhalation of silica
48
What is asbestosis?
fibrosis of lungs due to asbestos exposure
49
What is mesothelioma?
tumour of lining of the lung- mix of sarcoma & carcinoma cells
50
What is pathophysiology of occupational lung diseases (OLD)...
- 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
Triggers of OLD...
CWP: coal dust Silicosis: long term exposure to stone (e.g. sandblasting) Asbestosis: long term exposure to asbestos fibres e.g. building site
52
True/False: | Bent asbestos fibres are more dangerous that straight asbestos fibres?
False!
53
Presenting symptoms of CWP...
dyspnoea & dry cough, black sputum
54
Presenting symptoms of silicosis...
dyspnoea & dry cough
55
Presenting symptoms of asbestosis...
progressive dyspnoea & cough, clubbing, basal inspiratory crackles
56
Presenting symptoms of mesothelioma...
effusion, chest wall pain, dyspnoea, wt loss,
57
OLD ix...
PFTs, CXR, HRCT
58
CWP CXR findings..
many round opacities in upper zones
59
silicosis CXR findings..
thin streaks of calcification around hilar lymph nodes, opacities in upper lung
60
Asbestosis CXR findings...
honeycombing and streaky shadowing
61
Ix for mesothelioma...
thorascopic pleural biopsy, thoracentesis*, CXR
62
Tx for OLD..
CWP: avoid exposure, treat bronchitis Silicosis: avoid exposure Asbestosis: corticosteroids
63
True/False: | A complication of asbestosis is inc risk of cancer?
True
64
Tx for mesothelioma...
surgery, chemo, palliative, pleurodesis (obliteration of pleural cavity)
65
Prognosis for OLD?
All involve pulmonary fibrosis so bad prognosis
66
Prognosis for mesothelioma?
2 years
67
Is compensation available for OLD and for HP?
Yes!

Respiratory (19 decks)

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