IPF

Question 1

Where does IPF fit into the scheme of dz?

Answer 1

It is an idiopathic interstitial pneumonia (IIP) which is an interstitial lung disease (along with pneumoconioses, granulomatous dz, etc)

Question 2

What is the pathogenesis of IPF

Answer 2

Fibrosis initiates b/c of inflammation (due to recurrent microinjuries) but progresses in its absence

Question 3

Who gets IPF

Answer 3

pts >55 yo. mostly men b/c of exposure to occupational irritants. Smokers

Question 4

What is a UIP pattern on HRCT

Answer 4

subpleural and basal predominance or reticular abnormalities and honeycombing

Question 5

Not UIP on HRCT

Answer 5

upper or midlobe predominance, peribronchal predominance, extensive ground glass

Question 6

What type of biopsy is done?

Answer 6

surgical. Smaller biopsy doesnt always find it cause its patchy

Question 7

What does the biopsy for UIP show

Answer 7

patchy, fibroblastic foci, honeycombing

Question 8

What is the survival for IPF

Answer 8

less than five years but there are three possibilities: rapid, ipf w/ emphysema, slow with acure exacerbations

Question 9

What is the Tx for IPF?

Answer 9

lung translplant

Question 10

How do you diagnose IPF?

Answer 10

hx, HRCT, biopsy, EXCLUDE ALL OTHER POSSIBILITIES