Idiopathic Pulmonary Fibrosis Flashcards
What is idiopathic pulmonary fibrosis?
Chronic lung condition characterised by progressive fibrosis of the interstitutium of the lungs.
Who does idiopathic pulmonary fibrosis usually affect?
People aged 50-70
Usually males
How does idiopathic pulmonary fibrosis present?
- progressive exertional dyspnoea
- bibasal fine end-inspiratory crepitations on auscultation
- dry cough
- clubbing
What might you hear on auscultation in someone with IPF?
Bibasal fine inspiratory crepitations
How is idiopathic pulmonary fibrosis diagnosed?
High resolution CT
What might you find on spirometry of someone with idiopathic pulmonary fibrosis?
Restrictive picture
Reduced transfer factor
What might you see on an x-ray of someone with idiopathic pulmonary fibrosis?
- Bilateral interstitial shadowing- small, irregular, peripheral opacities
- ‘ground-glass’ - later progressing to ‘honeycombing
What is the main medical management of idiopathic pulmonary fibrosis?
Pirfenidone
What is the function of Pirfenidone?
Slows the progression of the disease
What is the only cure for idiopathic pulmonary fibrosis?
Lung transplant
What is the prognosis for idiopathic pulmonary fibrosis?
Poor
2-5 years
What would you see on spirometry of someone with IPF?
FEV1 in normal/decreased, FVC decreased, FEV1/FVC increased ⇒ shows restrictive lung disease
