Idiopathic pulmonary fibrosis

Question 1

Idiopathic pulmonary fibrosis
(Usual interstitial pneumonitis) presentation

Answer 1

The answer on USMLE for a patient over the age of 50 who has 6-12+ months of unexplained dry cough. This is how it shows up 4/5 times.

Vignette can also mention loud P2 means pulmonary hypertension) with “dry inspiratory crackles heard bilaterally.”

1/10 times, the Q will be patient over 50 with increasing fatigue and shortness of breath over 6-12 months, with only 1 month of cough, where it initially sounds like heart failure, and they’ll say CXR shows “interstitial markings” instead of reticular/reticulonodular patterning.
However, they say patient has “­ FEV1/FVC showing restrictive pattern” in the stem, which gives it away.

Question 2

Idiopathic pulmonary fibrosis
(Usual interstitial pneumonitis) PFTs

Answer 2

Textbook restrictive lung disease, with normal or ­increased FEV1/FVC. The reason for the FEV1/FVC being greater than in obstructive lung disease is radial traction

Question 3

Idiopathic pulmonary fibrosis
(Usual interstitial pneumonitis) imaging

Answer 3

CXR and CT scan show “reticular” or “reticulonodular” pattern.

These descriptors are exceedingly HY on USMLE, where students will overlook
them in the vignette, but they are hugely buzzy for restrictive lung disease. They are colloquially known as “honeycombing,” but I have not
seen the USMLE give a fuck about the latter colloquialism. They frequently just say “reticular” and “reticulonodular,” and then you know right away, “Boom. Restrictive lung disease,” i.e., fibrosis, etc.

Question 4

“Reticulogranular” finding on imaging. Dx?

Answer 4

“Reticulogranular” finding on imaging frequently for neonatal respiratory distress syndrome (NRDS)

Question 5

Idiopathic pulmonary fibrosis
(Usual interstitial pneumonitis) NBSM after the CXR and spirometry are performed

Answer 5

2CK wants “high-resolution CT of chest”

Question 6

Answer 6

Idiopathic pulmonary fibrosis
(Usual interstitial pneumonitis)
“Honeycombing” = reticular / reticulonodular pattern.

Question 7

Answer 7

Idiopathic pulmonary fibrosis
(Usual interstitial pneumonitis)

Question 8

Tx of on 2CK =

Answer 8

pirfenidone, anti-fibrotic agent that inhibits TGF-b-mediated synthesis of collagen

Question 9

To confirm diagnosis of interstitial lung disease (i.e., idiopathic pulmonary fibrosis) after imaging.

Answer 9

“lung biopsy”