Flashcards in L63 Deck (22):
What is interstitial lung disease aka diffuse lung disease?
Group of lung diseases w/ inflammation and/or fibrosis of the lung parenchyma
Might extend into small airways or alveoli
Usually restrictive PFTs - needed to det severity
What is the CC for ILD pts?
Dyspnea with activity and at rest
What types of things would cause an exogenous vs endogenous lung injury?
Exo = inhaled -> epithelial injury
Endo = internal issues (sepsis) -> endothelial injury
Idiopathic pulmonary fibrosis (IPF) is a type of ILD:
50-70 yrs old
Crackles +/- clubbing
Restrictive PFTs w/ low DLco = loss of functioning alveolar capillary units
What is the histo for idiopathic pulm fibrosis? Name 4 attributes you should be looking for.
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
What are the 4 CT findings for idio pulm fibrosis?
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
Do you need to biopsy to get IPF dx?
History + exam + CT+ PFT
Get biopsy for cancer, sarcoid, infection
Does a UIP pattern on radiology or histo mean you have IPF?
A UIP pattern can confirm IPF
But you can also get a UIP pattern for:
- CT disease
- Drug tox
No great options
Maybe corticosteroids - but not indicated for most pts
1. Nintedanib = X tyrosine kinase receptors
2. Pirfenidone = non-specific anti-fibrosis and anti-inflam
Outcomes for IPF pts who get a lung transplant
90% survive 1 year
50% survive 4 years
Non-specific interstitial pna is a type of ILD.
Dx w/ VATS lung biopsy
What is the histo pattern of NSIP?
= 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"
What is the CT pattern of NSIP?
Diffuse changes but the edges/pleura is spared
What is the prognosis and treatment for NSIP?
Prognosis better than IPF
Better response to CS (anti-inflam)
What is organizing pna?
Deposits of spindle shaped fibroblasts within the ECM
Located in the lumen of small airways or alveoli
What are the histo findings of organizing pna?
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
What is required to dx OP?
Biopsy -> histo
What does the CT look like for OP?
Consolidation, nodules, ground glass opacities
What is the main difference between a fibroblast foci (IPF) and organization (OP)?
Fibroblast focus (IPF) has background pattern of UIP * is sub-epi
Organization (OP) has preserved background pattern and is intra-luminal (small airways/alveoli)
Prognosis and treatment of OP
Prognosis better than IPF (similar to NSIP)
Better response to CS
If your chest CT has a UIP pattern with an identifiable etiology, what are you thinking?
UIP secondary to that etiology (CT disease, asbestos...)