Gomez - Diseases of Vascular Origin

Question 1

What is historically the classic test for diagnosing PE?

What is the current gold standard?

Answer 1

Historical: V/Q scan

Current: Pulmonary angiography

Question 2

What cutoff defines pulmonary HTN?

Answer 2

When pulmonary artery pressure ≥25mmHg

Question 3

What can cause WHO Group 1 pulmonary artery HTN?

Answer 3

Primary (idiopathic) pulmonary artery HTN

Autoimmune connective tissue disorders

drugs

Question 4

What can cause pulmonary HTN as a result of lung parenchymal disease or hypoxemia?

Answer 4

Chronic obstructive lung disease (i.e. bronchitis)

Chronic intersititial lung disease

Obstructive sleep apnea

Question 5

What is the role of BMPR2 in pulmonary HTN?

What happens when it is mutated?

Answer 5

BMPR2 inhibits proliferation of smooth muscle cells and favors apoptosis

When mutated, favors smooth muscle proliferation, causing thickening of muscularis layer and therefore decreasing lumen size, causing HTN

Question 6

Describe the progression of secondary pulmonary HTN

Answer 6

Increased pressure from any cause results in hypertrophy of tunica media

This hypertrophy increases pressure, causing further narrowing

Further narrowing causes increased pressure, leading to more tunica media hypertrophy

Eventually will result in cor pulmonale

Question 7

What is the pulmonary presentation of Goodpasture Syndrome?

Answer 7

Necrotizing hemorrhagic interstitial pneumonitis

Question 8

What is the mechanism behind Goodpasture syndrome?

Answer 8

Autoimmune attack

Anti-basement membrane antibodies to α3 chain of collagen IV

Question 9

What will a typical exam question concerning Goodpasture involve?

Answer 9

Presence of SOB and/or hemoptysis with elements of renal failure (i.e. elevated creatinine)

Question 10

What is the triad of Wegener granulomatosis?

What is the major abnormal laboratory finding?

Answer 10

Vasculitis/inflammation in sinuses, lungs, kidneys

c-ANCA typically found