Idiopathic pulmonary fibrosis

Question 1

what is the epidemiology of IPF?

Answer 1

more common in males

Question 2

what is the pathophysiology of IPF?

Answer 2

aberrant cellular repair
Pleura cobblestoned?
Stiffening of lung - due to chronic inflammation
Caring on lung tissue in later life
Fibroblasts become myofibroblasts in lungs - deposit collagen in extracellular matrix, becomes stiff, resistant to cell death, proliferate, form fibroblastic foci, thickened tissue leads to thickening of alveolar wall and destruction of alveoli
Special heterogeneity - normal lung next to damaged

Question 3

what are the key presentations of IPF?

Answer 3

Insidious onset 
Dry cough 
Fine crepitations 
Clubbing 
Cyanosis

Question 4

what is the appearance of IPF on histology?

Answer 4

Honeycomb cysts on microscopic histology, next to normal lung tissue
Usual interstitial pneumonia

Question 5

how is IPF treated?

Answer 5

Pirfenidone

Nintedanib

Question 6

what are the complications of IPF?

Answer 6

Side effects of pirfenidone - photosensitive skin, drug burden (3, 3 a day)

Question 7

what is the prognosis of IPF?

Answer 7

2-5 years from diagnosis survival