Idiopathic Pulmonary Fibrosis

Question 1

a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia. It causes lung scarring, which, over time, results in reduced oxygen intake.

Answer 1

Idiopathic Pulmonary Fibrosis

Question 2

Idiopathic Pulm Fibrosis

Whos most at risk for this?

Answer 2

Hairdressers, ranchers, farmers, stonecutters, metal workers

Question 3

Idiopathic pulm fibrosis

What does it sound like during PE?

Answer 3

fine, dry, inspiratory crackles (velcro crackles) at both lung bases

Question 4

Idiopathic pulm fibrosis

CXR Findings?

Answer 4

peripheral reticular opacities (netlike linear and curvilinear densities) mostly at lung bases. Honecombing (coarse reticular pattern) and lower lobe vol loss

Question 5

Idiopathic pulm fibrosis

WU

Answer 5

CXR
CT - more sensitive for honeycombing
PFT - restrictive
6 min walk test

Question 6

Rx for idiopathic pulm fibrosis

Answer 6

💊Pirfenidoneornintedanib - antifibrotic drugs that slow progression

Question 7

idiopathic pulm fibrosis

trmnt

Answer 7

1. refer to pulm
2. pirfenidone or nintedanib
3. O2
4. Pulm rehab
5. lung transplant sometimes
6. vacc for flu, pneumococcal
7. smoking cessation

Question 8

idiopathic pulm fibrosis

prognosis

Answer 8

3 yrs after dx
resp failure -> death
end of life planning!