Lecture 64 - Diffuse (Parenchymal) Lung disease

Question 1

IPF

• cause?
• prognosis?
• PFT pattern?
• Histological pattern?

Answer 1

Etiology – Idiopathic

Poor survival – Median survival is 2-4 years

PFT – restrictive

Histological pattern – Usual Interstitial PNA – (UIP)

Question 2

Chest CT Findings of IPF?

mild

severe

occasional additional finding/complication

lobar predominance?

Answer 2

mild – Peripheral, Subpleural Opacities, reticular

severe – Subpleural cysts (honey combing) — more severe (involves central lung parenchyma)

ocassional additional finding – traction bronchiectasis

Lower Lobe Predominance

Question 3

Pathology/Histology: Usual Interstitial PNA (UIP) Pattern –

low power?
defining characteristic of high power?

Answer 3

Lower Power –
lots of dilated spaces — correlates with honey combing;
Patchy, non-uniform areas of collagen accumulation Geographic Heterogeneity

Higher Power – Fibroblast focus

Question 4

Treatment of IPF?

Answer 4

O2 – supportive
Best Treatment: Lung Transplant

no use for steroids or bronchodilators

Question 5

Non Specific Interstitial PNA (NSIP)

clinical name?
histological pattern?
prognosis compared to IPF?

Answer 5

Clinical: NSIP –

	Histological: NSIP 

Outcomes: Better Prognosis than IPF

Question 6

NSIP – describe the histological findings

Answer 6

Diffusely thickened, but still maintains architecture of the lung

Nonspecific thickening of the alveolar - capillary membrane
Interstitial inflammation with mild fibrosis

no honey combing
no fibroblast foci

Question 7

NSIP – describe the CT findings

Answer 7

Imaging: CT –
Diffuse reticulation;
No peripheral predilection
Usually sparing of the periphery

no honey combing

Question 8

NSIP treatment options?

Answer 8

Treatment:

Better response to corticosteroids, in general

Question 9

Organizing PNA –
clinical term?
histological term?

Answer 9

clinical and histological: Organizing PNA

Question 10

Organizing PNA –

histo findings:

Answer 10

Multiple Foci of organizing Lung injury on a background on a relatively preserved lung architecture
The organization Foci are patchy;

Not diffuse (like DAD)

Question 11

Organizing PNA –

CT Findings

Answer 11

Consolidation in a patchy pattern

Sparing of the periphery

Question 12

IPF histo vs Organizing PNA histo

Answer 12

Organizing PNA: Multiple foci of organization on a relatively preserved background lung architecture;

IPF: Foci of Organization on background on UIP;