THORACIC Section 7: Cystic Lung Disease

Question 1

Cystic lung disease associated with a young (20s-30s) smoker

Answer 1

Pulmonary Langerhans Cell Histiocystosis (LCH)

Question 2

Cystic lung disease associated wiht Child bearing aged women & Tuberous sclerosis

Answer 2

Lymphangiomyomatosis (LAM)

Question 3

Cystic lung disease associated with renal findings (bilateral oncocytomas and chromophobe RCCs).

Answer 3

Birt Hogg Dube (BHD)

Question 4

Cystic lung disease associated with a bunch of gross skin stuff

Answer 4

Birt Hogg Dube (BHD)

Question 5

Cystic lung disease with a pathophysiology that is Estrogen dependent (Why it srongly favors women)

Answer 5

Lymphangiomyomatosis (LAM)

Question 6

A. Cystic lung disease associated with:
1. Seizures
2. Mental Retardation
3. Adenoma Sbaceum.
(SSS: Seizures, Sebaceum, Stupid)

This triad is suggestive of?

Answer 6

A. Lympahngiomayomatosis.

B. Tuberous sclerosis

Question 7

Cystic lung disease showing kidneys with multiple Angiomyolipoma (AML)

Answer 7

Lymphangiomyomatosis (LAM)

Question 8

What is this?
Descibe the cysts.

Answer 8

Langherhans Cell Histiocytosis (LCH)

The cysts are Thicker and Bizarre shaped - occurs when 2 ormore cysts merge together
Upper/Mid zone predominent
Spares the CP angles

Question 9

What is this?

Describe the cysts.

Answer 9

Lymphangiomyomatosis (LAM)

Thin-walled ROUND cysts, Uniform or “Diffuse” distribution

Question 10

What is this?

Describe the cysts

Answer 10

Birt Hogg Dube (BHD)

Thin-walled OVAL “Floppy” cysts
Lower Zone predominant and favor the paramediastinal regions

Question 11

Cystic lung disease associated with chylothorax

Answer 11

Lymphangiomyomatosis (LAM)

Question 12

Cystic lung disease that is a benign lymphoproliferative disorder with infiltration of the lungs.

Answer 12

Lymphocytic Interstitial Pneumonitis (LIP)

Question 13

Cystic lung disease associated wiht Autoimmune diseases

Answer 13

Lymphocytic Interstitial Pneumonitis

Question 14

Autoimmunie disease which is concomitant in 25% of Lymphocytic Interstitial Pneumonitis (LIP) cases

Answer 14

Sjogrens

Question 15

Associated autoimmune disease in YOUNG patients with Lymphocytic Interstitial Pneumonitis (LIP)

Answer 15

HIV

Question 16

Autoimmune diseases associated with Lymphocytic Interstitial Pneumonitis (LIP)

Answer 16

SLE
RA
Sjogrens
Castlemans

Question 17

What is this?

Descibe the cysts

Answer 17

Lymphocytic Interstitial Pneumonitis (LIP)

Thin-walled cysts with ground glass or nodules
“Deep within the lung parenchyma”
Seen predominantly with Sjogrens

Question 18

When I say LIP … Think of? 1. ___ 2. ____
When I say LIP in a kid, think of? ___.
When I say “Perivascular cysts”, you say? ____

Answer 18

When I say LIP … Think of? 1. Sjogrens and 2. HIV.
When I say LIP in a kid, think of HIV.
When I say “Perivascular cysts”, you say? LIP

Question 19

Often has the descripto of “Perivascular cysts” where multiple vessles seen coursing alon gthe cysts.

Answer 19

Lymphocytic Interstitial Pneumonitis (LIP)

Question 20

The most common opportunistic infection in AIDS.

Answer 20

Pneumicystis Pneumonia (PCP)

Question 21

Typical appearance of Pneumocystis Pneumonia (PCP)

Answer 21

“Ground glass appearance predominantly in teh hilar and mid lung zones”

Question 22

What is this?

Describe

Answer 22

Pneumocystis Pneumonia (PCP)

Apical predomina thin walled cysts wiht GROUND GLASS in the mid-lung/peri-hilar regions

Question 23

AIDS + Ground Glass Lungs

Think of?

Answer 23

PCP

Question 24

AIDS + Pneumothorax?

Answer 24

PCP

Question 25

Idiopathic cause of giant bullous emphysema, resulting from avascular necrosis of the lung parenchyma and hyperinflation.

Answer 25

Vanishing lung syndrome

Question 26

A bullous disease occyuping at least 1/3 of a hemithorax favoring bilatearl upper lobes

Answer 26

Vanishin Lung Syndrome

Question 27

Complication of Vanishing Lung syndrome

Answer 27

Tension pneumothorax

Question 28

Young male
Smoker
Alpha-1 antitrypsin deficiency

Answer 28

Vanishing lung syndrom risk factors