Vascular Pulmonary Disease

Question 1

What are primary and secondary causes of a hypercoagulable state that can result in pulmonary emboli?

Answer 1

Primary: SLE
Secondary: Cancer

Question 2

How does a patient present with PE in tested and physical exam?

Answer 2

Pulseless electrical activity

EKG shows a rhythm

Question 3

Main diagnostic tool for PE.

Answer 3

CT angiography

-opacities appear in lung fields due to inflammation and/or hemorrhage

Question 4

What can appear on CXR indicating PE?

Answer 4

Hampton’s Hump

Question 5

Type of necrosis that occurs when lung tissue becomes ischemic due to PE.

Answer 5

Coagulative Necrosis

-architecture is maintained

Question 6

How can you tell if an embolus formed pre or post mortem?

Answer 6

Lines of Zahn

-microscopic characteristic of a clot formed in active blood flow

Question 7

Cell that always arrives during hemorrhage.

Answer 7

Macrophages

-phagocytose the hemorrhaged RBCs, then the iron from the heme is visible (hemosiderin-laden macrophages)

Question 8

Major cause of fat emboli

Answer 8

Long Bone Fractures

Question 9

What is classified as pulmonary HTN?

Answer 9

Mean Pulmonary Artery pressure greater than:
25mmHg at rest
30mmHg with exercise

Question 10

Two major causes for primary Pulmonary HTN.

Answer 10

1. Hereditary

2. Drug Induced: Fen-Phen, cocaine, amphetamines

Question 11

How does primary Pulmonary HTN appear on X-ray or CT?

Answer 11

RV hypertrophy, increased cardiothoracic ration, dilation of the pulmonary arteries

Question 12

What are characteristics of reversible damage caused to pulmonary blood vessels in Pulmonary HTN?

Answer 12

Medial Hypertrophy

Intimal Thickening or Proliferation

Question 13

What are characteristics of irreversible damage caused to pulmonary blood vessels in Pulmonary HTN?

Answer 13

Concentric Laminar Intimal Fibrosis
Fibrinoid Necrosis
Plexiform Lesions (looks like a glomerulus 90 degrees off a vessel)

Question 14

Where do atheromas commonly form during pulmonary HTN?

Answer 14

Pulmonary arteries and major branches

Question 15

What congenital defect commonly leads to pulmonary HTN?

Answer 15

ASDs

-increase the pressure on the right side of the heart and increase pressure in the lungs

Question 16

What is the triad in Goodpasture Syndrome?

Answer 16

Diffuse Pulmonary Hemorrhage
Glomerulonephritis
Anti-glomerular basement membrane Abs

Question 17

Treatment for Goodpasture Syndrome.

Answer 17

Flushing the plasma to remove the autoimmune Abs

Question 18

What microscopic findings are seen in the lungs and kindeys in Goodpasture Syndrome?

Answer 18

Lungs

• FOCAL necrosis
• fibrous thickening and hyperplasia of alveolar septa
• linear Ig deposits on basement membrane

Kidneys

• FOCAL crescentic glomerulonephritis
• linear deposits of Ig and complement on basement membranes

Question 19

What is the difference between Idiopathic Pulmonary Hemosiderosis and Goodpastures or Wegeners?

Answer 19

Children and Teens with intermittent hemoptysis
No circulating Abs or renal involvement
No necrosis
Hemosiderin Laden Macrophages

Question 20

Possible etiology of Wegener Syndrome

Answer 20

Antineutrophil cytoplasmic antibodies

-directed against neutrophil proteinase 3

Question 21

What are the microscopic findings seen in the lungs and kidneys in Wegener Syndrome?

Answer 21

Lungs

• necrotizing granulomas
• DIFFUSE arteritis (not focal)

Kidneys
-necrotizing glomerulonephritis