idiopathic pulmonary fibrosis Flashcards

1
Q

What does restrictive/interstitial disease affect and cause?

A

affects lung parenchyma
->causing inflammation and fibrosis

-> leading to reduced lung compliance

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2
Q

What is idiopathic pulmonary fibrosis?

A

it is an interstitial/restrictive lung disease with no known cause
-> leads to deterioration in lung function

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3
Q

What is fibrosis?

A

Replacement of normal elastic and functional lung tissue with scar tissue that’s stiff and doesn’t function properly

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4
Q

what is idiopathic pulmonary fibrosis associated with?

A

High-risk of lung cancer
- due to dysplastic epithelial changes in fibrosis
-> leading to invasive malignancy

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5
Q

what are 5 presentations of idiopathic pulmonary fibrosis?

A

60 - 70 years old, commonly in men

clubbing

right heart failure

chronic breathlessness and cough

fine end inspiratory crepitations (crackles)

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6
Q

What are 6 signs of interstitial lung disease?

A
  • Dyspnoea on exertion
  • low FEV
  • low FVC
  • hypoinflation due to stiffness
  • reduced CO gas transfer
  • reduced lung compliance
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7
Q

what are two investigations used to diagnose idiopathic pulmonary fibrosis?

A

Spirometry shows low, FEV one and load FVC et cetera.

High-resolution CT of thorax ground-glass appearance honeycombing, supportive, E.G.02, rehab, smoking, cessation all anti-fibrotic.

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8
Q

what are 3 treatments used for idiopathic pulmonary fibrosis?

A

supportive
e.g. O2 therapy, rehab, smoking cessation

oral anti-fibrotics e.g. pirfenidone and nintedanib

lung transplant

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