L63

Question 1

What is interstitial lung disease aka diffuse lung disease?
Cause
PFT results

Answer 1

Group of lung diseases w/ inflammation and/or fibrosis of the lung parenchyma
Might extend into small airways or alveoli
UNKNOWN CAUSE
Usually restrictive PFTs - needed to det severity

Question 2

What is the CC for ILD pts?

Answer 2

Dyspnea with activity and at rest

Question 3

What types of things would cause an exogenous vs endogenous lung injury?

Answer 3

Exo = inhaled -> epithelial injury
Endo = internal issues (sepsis) -> endothelial injury

Question 4

Idiopathic pulmonary fibrosis (IPF) is a type of ILD:
Age grp
PE findings
PFTs

Answer 4

50-70 yrs old
Crackles +/- clubbing
Restrictive PFTs w/ low DLco = loss of functioning alveolar capillary units

Question 5

What is the histo for idiopathic pulm fibrosis? Name 4 attributes you should be looking for.

Answer 5

Usual interstitial pna = UIP

• Patchy collagen (scar)
• Heterogeneity = fibrosis next to normal lung
• Honeycombing = enlarged air spaces lined by bronchiolar epithelium (could be filed with mucin)
• Fibroblast foci

Question 6

What are the 4 CT findings for idio pulm fibrosis?

Answer 6

1. Peripheral/subpleural opacities
2. Honeycombing (esp in lower lobes)
3. Lower lobe > upper lobe
4. Traction bronchiectasis
   NOT see consolidation, masses, or ground glass

Question 7

Do you need to biopsy to get IPF dx?

Answer 7

No
History + exam + CT+ PFT
Get biopsy for cancer, sarcoid, infection

Question 8

Does a UIP pattern on radiology or histo mean you have IPF?

Answer 8

NO
A UIP pattern can confirm IPF
But you can also get a UIP pattern for:
- CT disease
- Asbestos 
- Drug tox

Question 9

Treat IPF

Answer 9

No great options
Supportive O2
Maybe corticosteroids - but not indicated for most pts
NO bronchodilators
EXPENSIVE:
1. Nintedanib = X tyrosine kinase receptors
2. Pirfenidone = non-specific anti-fibrosis and anti-inflam

Question 10

Outcomes for IPF pts who get a lung transplant

Answer 10

90% survive 1 year

50% survive 4 years

Question 11

Non-specific interstitial pna is a type of ILD.

How dx

Answer 11

Dx w/ VATS lung biopsy

Question 12

What is the histo pattern of NSIP?

Answer 12

= NSIP (same name for clinical and histo)
Might look OK under low power
VERY thick septa make of lots of cells or collagen
“Interstitial inflammation and mild fibrosis”

Question 13

What is the CT pattern of NSIP?

Answer 13

Diffuse changes but the edges/pleura is spared

Question 14

What is the prognosis and treatment for NSIP?

Answer 14

Prognosis better than IPF

Better response to CS (anti-inflam)

Question 15

What is organizing pna?

Answer 15

Deposits of spindle shaped fibroblasts within the ECM

Located in the lumen of small airways or alveoli

Question 16

What are the histo findings of organizing pna?

Answer 16

“Organization”
Multiple foci of organization (likely patchy distribution aka diffuse disease)
Preserve background lung architecture
= Non-specific response to tissue injury, so this is a healing mechanism

Question 17

What is required to dx OP?

Answer 17

Biopsy -> histo

CT

Question 18

What does the CT look like for OP?

Answer 18

Consolidation, nodules, ground glass opacities

Periphery spared

Question 19

What is the main difference between a fibroblast foci (IPF) and organization (OP)?

Answer 19

Fibroblast focus (IPF) has background pattern of UIP * is sub-epi
Organization (OP) has preserved background pattern and is intra-luminal (small airways/alveoli)

Question 20

Prognosis and treatment of OP

Answer 20

Prognosis better than IPF (similar to NSIP)

Better response to CS

Question 21

If your chest CT has a UIP pattern with an identifiable etiology, what are you thinking?

Answer 21

UIP secondary to that etiology (CT disease, asbestos…)

Question 22

If your chest CT has a UIP pattern w/o a cause, what are you thinking?

Answer 22

UIP -> IPF