Idiopathic pulmonary fibrosis

Question 1

What is idiopathic pulmonary fibrosis?

Answer 1

Chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs. There are many causes of lung fibrosis (e.g. medications, connective tissue disease, asbestos) the term IPF is reserved when no underlying cause exists

Question 2

What are the clinical features of idiopathic pulmonary fibrosis?

Answer 2

progressive exertional dyspnoea
bibasal fine end-inspiratory crepitations on auscultation
dry cough
clubbing

Question 3

How is idiopathic pulmonary fibrosis diagnosed?

Answer 3

(spirometry) restrictive picture
(reduced transfer factor (TLCO) impaired gas exchange
(imaging) bilateral interstitial shadowing

Question 4

What is the treatment for idiopathic pulmonary fibrosis?

Answer 4

Pulmonary rehabilitation
Pirfenidone (an antifibrotic agent) may be useful in selected patients
Supplemental oxygen
Lung transplant