Idiopathic Pulmonary Fibrosis Flashcards
What is idiopathic pulmonary fibrosis?
A chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs
What age of people are commonly affected by idiopathic pulmonary fibrosis?
50-70 year olds
Does idiopathic pulmonary fibrosis more commonly affect men or women?
Men 2x
How does Idiopathic Pulmonary Fibrosis
commonly present?
progressive exertional dyspnoea
bibasal fine end-inspiratory crepitations on auscultation
dry cough
clubbing
What would you hear on auscultation of someone with idiopathic pulmonary fibrosis?
Bibasal fine end-inspiratory crepitations on auscultation
What investigation is required for the diagnosis of idiopathic pulmonary fibrosis?
CT chest
What would you see on a CT chest of someone with idiopathic pulmonary fibrosis?
Reticulonodular shadowing and Honeycomb lung
What other investigations can you do for someone with idiopathic pulmonary fibrosis?
Spirometry
Chest x-ray
What would you see on a chest x-ray of someone presenting with idiopathic pulmonary fibrosis?
Bilateral lower zone reticulo-nodular shadowing
What picture would you see on spirometry of someone with idiopathic pulmonary fibrosis?
A restrictive picture
–FEV1 normal/decreased
–FVC decreased
–FEV1/FVC increased
What else would be reduced in someone with idiopathic pulmonary fibrosis?
Reduced transfer factor
What is the conservative management of someone with idiopathic pulmonary fibrosis?
Pulmonary rehabilitation (main)
Smoking cessation
What is the medical management of idiopathic pulmonary fibrosis?
Pirfenidone- an antifibrotic agent (some evidence)
What else might a patient with idiopathic pulmonary fibrosis require?
Long Term oxygen therapy
Lung transplant
What is the prognosis of idiopathic pulmonary fibrosis?
Poor, average life expectancy is around 3-4 years
