idiopathic pulmonary fibrosis Flashcards
What does restrictive/interstitial disease affect and cause?
affects lung parenchyma
->causing inflammation and fibrosis
-> leading to reduced lung compliance
What is idiopathic pulmonary fibrosis?
it is an interstitial/restrictive lung disease with no known cause
-> leads to deterioration in lung function
What is fibrosis?
Replacement of normal elastic and functional lung tissue with scar tissue that’s stiff and doesn’t function properly
what is idiopathic pulmonary fibrosis associated with?
High-risk of lung cancer
- due to dysplastic epithelial changes in fibrosis
-> leading to invasive malignancy
what are 5 presentations of idiopathic pulmonary fibrosis?
60 - 70 years old, commonly in men
clubbing
right heart failure
chronic breathlessness and cough
fine end inspiratory crepitations (crackles)
What are 6 signs of interstitial lung disease?
- Dyspnoea on exertion
- low FEV
- low FVC
- hypoinflation due to stiffness
- reduced CO gas transfer
- reduced lung compliance
what are two investigations used to diagnose idiopathic pulmonary fibrosis?
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.
what are 3 treatments used for idiopathic pulmonary fibrosis?
supportive
e.g. O2 therapy, rehab, smoking cessation
oral anti-fibrotics e.g. pirfenidone and nintedanib
lung transplant