idiopathic pulmonary fibrosis

Question 1

define idiopathic pulmonary fibrosis

Answer 1

Formation of scar tissue in lungs with no known cause.

Question 2

what is the most common type of interstitial lung disease

Answer 2

idiopathic pulmonary fibrosis

Question 3

risk factors for idiopathic pulmonary fibrosis

Answer 3

• smoking
• occupational (e.g. dust)
• drugs (methotrexate)
• viruses (EBV,CMV)

Question 4

who does idiopathic pulmonary fibrosis usually present in

Answer 4

Seen in older men (60+) who smoke

Question 5

pathophysiology of idiopathic pulmonary fibrosis

Answer 5

progressive scarring leading to type 1 respiratory failure

Question 6

symptoms of idiopathic pulmonary fibrosis

Answer 6

• Dyspnoea
• dry unproductive cough
• bibasal crackles

Question 7

2 investigations for idiopathic pulmonary fibrosis

Answer 7

1. spirometry
2. high resolution CT - GOLD STANDAD

Question 8

how to do spirometry for pulmonary fibrosis

Answer 8

restrictive lung disease
FEV1:FVC > 0.7
BUT FVC is low (<0.8)

Question 9

what does CT for pulmonary fibrosis show

Answer 9

GROUND GLASS appearance of lungs

Question 10

pharmacological treatment for pulmonary fibrosis

Answer 10

Pirfenidone, nintedanib

Question 11

non pharmacological treatment for pulmonary fibrosis

Answer 11

• smoking cessation
• physiotherapy
• vaccines up to date
• lung transplantation last resort