Idiopathic Pulmonary Fibrosis (pathology)

Question 1

What is idiopathic pulmonary fibrosis?

Answer 1

Lungs become scarred and breathing becomes difficult

Question 2

Causes of IPF

Answer 2

MOSTLY IDIOPATHIC

• Age
• Male
• Connective tissue disease (scleroderma, rheumatoid disease)
• Drug reaction
• Post infection
• Asbestos

Question 3

Symptoms and signs of IPF

Answer 3

• Chronic SOB
• Cough
• Clubbing
• Crackles

Predisposes to lung cancer

Question 4

Investigations of IPF

Answer 4

CXR: 
-	Small lungs 
CT: 
-	Honeycombing 
Restrictive spirometry 
Reduced gas transfer

Question 5

Median survival of IPF

Answer 5

3 years

Question 6

Medical and surgical treatment of IPF

Answer 6

Medical: 
-	OAF oral anti fibrotic – slow down progression but does NOT stop it 
-	palliative care 
Surgical: 
-	Transplant