Idiopathic Pulmonary Fibrosis

Question 1

Which groups are most commonly affected by IPF?

Answer 1

Smokers
Elderly

Question 2

What is the pathology responsible for IPF?

Answer 2

Repetitive injury of the alveolar epithelium by an unknown agent, meaning repair mechanisms are activated. However, these spiral out of control, leaving fibrosis behind.

This leaves very dilated spaces behind. Often termed a ‘honeycomb’ appearance.

Question 3

What is a typical presentation of IPF?

Answer 3

Progressive dyspnoea
Dry cough
Weight loss
Fatigue
Malaise
Clubbing
Cyanosis
Bilateral fine inspiratory crackles

Question 4

How would IPF affect spirometry?

Answer 4

Would have restrictive pattern (low FEV1, and low FVC).

Question 5

How would IPF present on a CXR?

Answer 5

Bilateral lower zone reticulonodular shadowing
Bilateral infiltration
Reduced lung volume

Question 6

How would IPF present on a CT scan?

Answer 6

Ground glass appearance (early-stage)
Honeycombing (late-stage)

Question 7

How is IPF treated?

Answer 7

Give oxygen therapy if hypoxic
Treat with anti-fibrotics to slow progression
If patient is young, lung transplant may be indicated

Question 8

What is an example of an anti-fibrotic drug?

Answer 8

Nintedanib