Idiopathic pulmonary fibrosis

Question 1

What is interstitial lung disease?

Answer 1

A group of conditions that cause inflammation and fibrosis of the lung parenchyma

Question 2

What conditions are under interstitial lung disease?

Answer 2

Idiopathic pulmonary fibrosis
Secondary pulmonary fibrosis
Hypersensitivity pneumonitis
Asbestosis

Question 3

What is the presentation of idiopathic pulmonary fibrosis

Answer 3

Shortness of breath on exertion
Dry cough
Fatigue

Question 4

What signs do patients with idiopathic pulmonary fibrosis have on examination?

Answer 4

Bibasal fine end-inspiratory crackles
Finger clubbing

Question 5

How is IPF diagnosed?

Answer 5

Spirometry
Reduced transfer factor (TLCO)
CXR - bilateral reticulonodular opacification (ground glass)
CT

Question 6

What spirometry results are seen in IPF?

Answer 6

Restrictive picture
- FEV1 normal/decreased
- FVC decreased
- FEV1:FVC > 80% normal

Question 7

What is the investigation of choice for diagnosis of IPF?

Answer 7

High resolution CT thorax

Question 8

What is the management of IPF?

Answer 8

Pulmonary rehabilitation
Smoking cessation
Home oxygen (if hypoxic)
Pneumococcal and flu vaccines
Advanced care planning

Question 9

Which medications can slow the progression of IPF?

Answer 9

Pirfenidone
Nintedanib
- These are used when FVC is 50-80 of predicted