Idiopathic pulmonary fibrosis

Question 1

Idiopathic pulmonary fibrosis - in a few words

Answer 1

Interstitial pneumonia, inflammatory cell infiltrate leading to pulmonary fibrosis

Question 2

Pathophysiology

Answer 2

Alveolar lining damaged
TGF-B1 released by type II pneumocytes
TGF-B1 makes fibroblasts proliferate and develop into myofibroblasts
These secrete reticular fibres and collagen

The problem is when there become too many type II pneumocytes :. myofibroblasts :. collagen

Question 3

Symptoms

Answer 3

Dry cough
Dyspnoea o/e
malaise
weight loss
arthralgia

Question 4

Signs

Answer 4

Cyanosis
Finger clubbing
fine end inspiratory crepitations
CLUBBING!

Question 5

Tests

Answer 5

Spirometry - restrictive pattern (FEV down, FVC down FEV/FVC ratio normal)
ABG
CRP up
immunoglobulins up
ANA positive in 30%
Lung biopsy

Question 6

Treatment

Answer 6

Supportive/palliative
O2
Opioids
Transplant

Question 7

Can you get finger clubbing in COPD?

Answer 7

Ecclesiastes 7:29 This is all that I have learned: God made us plain and simple, but we have made ourselves very complicated.

Finger clubbing is not a sign of COPD.
However, a bit of fibrosis in COPD is not unheard of - so you could get a bit of IPF in the background of COPD

So no but yes. But no.

Question 8

Remind me what pattern we see on spirometry?

And what will happen to lung capacity?

Answer 8

Restrictive pattern, reduced lung capacity