Idiopathic Pulmonary Fibrosis

Question 1

What is IPF?

Answer 1

Condition characterised by progressive fibrosis of the interstitium of the lungs when there is no obvious underlying cause.

Question 2

What is the epidemiology of IPF?

Answer 2

Men 2:1

50-70 yo

Question 3

What are the features of IPF?

Answer 3

Progressive exertional dyspnoea
Bibasal fine end-inspiratory crepitations
Dry cough
Clubbing

Question 4

How is IPF diagnosed?

Answer 4

Spirometry
Reduced TLCO: impaired gas exchange
High-resolution CT for diagnosis ‘honeycombing’
Chest x-ray bilateral interstitial shadowing ‘ground-glass’
ANA positive in 30%

Question 5

What picture is seen on spirometry in IPF?

Answer 5

Restrictive picture
FEV 1 normal or decreased
FVC markedly decreased
FEV1/FVC increased

Question 6

What is the managemeent of IPF?

Answer 6

Pulmonary rehabilitation
Pirfenidone
Supplementary O2

Question 7

What is the prognosis of IPF?

Answer 7

Poor, avarage life expectancy of 3-4 years.