idiopathic pulmonary fibrosis

Question 1

What does restrictive/interstitial disease affect and cause?

Answer 1

affects lung parenchyma
->causing inflammation and fibrosis

-> leading to reduced lung compliance

Question 2

What is idiopathic pulmonary fibrosis?

Answer 2

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

Question 3

What is fibrosis?

Answer 3

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

Question 4

what is idiopathic pulmonary fibrosis associated with?

Answer 4

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

Question 5

what are 5 presentations of idiopathic pulmonary fibrosis?

Answer 5

60 - 70 years old, commonly in men

clubbing

right heart failure

chronic breathlessness and cough

fine end inspiratory crepitations (crackles)

Question 6

What are 6 signs of interstitial lung disease?

Answer 6

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

Question 7

what are two investigations used to diagnose idiopathic pulmonary fibrosis?

Answer 7

Spirometry shows low, FEV one and load FVC et cetera. High resolution CT of thorax groundglass appearance honeycombing , supportive, E.G.02, rehab, smoking, cessation all anti-fibrotic e.g. per phone The done professionally done per Finedon and tan lung transplant

Question 8

what are 3 treatments used for idiopathic pulmonary fibrosis?

Answer 8

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

oral anti-fibrotics e.g. pirfenidone and nintedanib

lung transplant