Interstitial lung disease Flashcards

1
Q

Restrictive extra-pulmonary causes of dyspnoea (neuromuscular)

A

diaphragmatic paralysis
cervical spine injury
myasthenia gravis
guillain-barre
muscular dystrophies

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2
Q

Restrictive extra-parenchymal causes of dyspnoea (chest wall)

A

kyphoscoliosis
obesity
ankylosing spondylitis

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3
Q

What does DPLD stand for?

A

diffuse parenchymal lung disease
(interstitial lung diseases)

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4
Q

What is the interstitium?

A

area in and around the alveoli

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5
Q

What can cause interstitial lung disease?

A

drugs, therapies, iatrogenic (bleomycin, amiodarone)
inhalation of dusts
connective tissue diseases
idiopathic

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6
Q

Name 2 ILD granulomatous diseases

A

sarcoidosis
hypersensitivity pneumonitis

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7
Q

Breath sounds in fibrosis

A

fine end inspiratory crackles (louder towards end of inspiration)

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8
Q

Breath sounds in sarcoidosis

A

normal

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9
Q

Breath sounds in hypersensitivity pneumonitis

A

crackles, wheeze, squeaks

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10
Q

How is exercise capacity measured?

A

6MWD = 6 minute walk distance = distance walked in 6 minutes and oxygen saturations before and after

1 minute sit-to-stand test = how many sit-to-stands in 1 minute and oxygen saturations before an after

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11
Q

What enzyme can be a marker for sarcoidosis?

A

ACE
Angiotensin-Converting Enzyme

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12
Q

Where does Ankylosing Spondylitis associated pulmonary fibrosis affect?

A

upper lobes

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13
Q

HRCT findings interstitial lung disease/fibrosis

A

ground glass (non-specific)
consolidation
reticular shadowing (fibrosis)
traction bronchiectasis (scar tissue pulls airways apart) (fibrosis)
honeycombing (fibrosis)

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14
Q

How can disease progression be monitored in ILD?

A

FVC
DLCO (diffusion capacity of lungs for carbon monoxide)
6 minute walk testing
symptoms - SOB, cough

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15
Q

ILD treatment

A

fibrosis:
- antifibrotics - only for IPF

inflammation:
- corticosteroids
- MTX/HCQ
- azathioprine/mycophenolate mofetil
- cyclophosphamide

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16
Q

IPF management

A

oxygen
lung transplant
pirfenidone/nintedanib (when FVC 50-80% predicted)
pulmonary rehab

17
Q

Pirfenidone MOA

A

reduced fibroblast proliferation
inhibits production of TGF-beta and collagen stimulation

18
Q

Pirfenidone side effects

A

nausea
photosensitive rash

19
Q

Nintedanib MOA

A

triple growth factor receptor inhibitor
- platelet-derived growth factor receptor
- fibroblast growth factor receptor
- vascular endothelial growth factor receptor

20
Q

Nintedanib side effects

A

diarrhoea - can be unannounced and severe

21
Q

What us the other name for hypersensitivity pneumonitis?

A

extrinsic allergic alveolitis

22
Q

What is observed on lung biopsies of hypersensitivity pneumonitis?

A

granulomas

23
Q

What can cause hypersensitivity pneumonitis?

A

organic dusts
- avian proteins = bird fancier’s lung
- moulds/fungi = farmer’s lung

24
Q

What does asbestosis increase the risk of?

A

pleural cancer (mesothelioma)
lung cancer