Vascular lung diseases

Question 1

Primary hypercoagulable states

Answer 1

Factor V Leiden mutation
Antiphospholipid antibody syndrome
Prothrombin mutation

Question 2

Secondary hypercoagulable states

Answer 2

Obesity
OCP use
Pregnancy
Recent surgery
Cancer

Question 3

Mechanism of increased pulmonary vascular resistance in PE

Answer 3

Vascular obstruction
Vascular constriction mediated by thromboxane A2 and serotonin

Question 4

Mechanism of hypoxemia in PE

Answer 4

Increased perfusion of lung unites with low ventilation (V/Q mismatch)

Decreased CO with decrease in mixed venous PO2

Question 5

Mechanism of increased alveolar dead space in PE

Answer 5

Vascular obstruction
Absent perfusion with preserved ventilation (V/Q mismatch)

Question 6

Mechanism of decreased lung compliance in PE

Answer 6

Loss of surfactant causing alveolar edema and hemorrhage

Question 7

Mechanism of increased airway resistance in PE

Answer 7

Reflex bronchoconstriction

Question 8

Mechanism of increased respiratory rate in PR

Answer 8

Reflex stimulation of irritant receptors

Question 9

Measurement of a degradation product of cross-linked fibrin

Answer 9

D-dimer

Question 10

ECG findings in PE

Answer 10

S1Q3T3 pattern
Sinus tachy

Question 11

Describe S1Q3T3 pattern on EKG

Answer 11

Large S wave in lead I
Q wave in lead III
Inverted T wave in lead III

Question 12

Complications of lung infarct

Answer 12

Septic infarcts
Abscess

Question 13

Mutation associated with idiopathic pulmonary HTN

Answer 13

BMPR2

Question 14

Function of BMPR2

Answer 14

Binds to TGF-beta, BMP, activin, and inhibin. Leads to dysfunction and proliferation of endothelial cells and vascular smooth muscle cells

Question 15

Inheritance of BMPR2 mutation

Answer 15

Autosomal dominant

Question 16

Typical demographic for idiopathic pulmonary HTN

Answer 16

Female between 20-40 yo

Question 17

Mechanism of secondary pulmonary HTN in chronic obstructive or interstitial lung diseases

Answer 17

Obliterate alveolar capillaries and increase pulmonary resistance

Question 18

Grade 1 vascular changes in pulmonary HTN

Answer 18

Medial hypertrophy of the pulmonary muscular and elastic arteries

Question 19

Grade 2 vascular changes in pulmonary HTN

Answer 19

Intimal and medial hypertrophy

Question 20

Grade 3 vascular changes in pulmonary HTN

Answer 20

Pipestem fibrosis of arteries

Question 21

Grade 4 vascular changes in pulmonary HTN

Answer 21

Plexiform lesions –> tuft of capillary formations

Question 22

CXR findings in pulmonary HTN

Answer 22

Enlarged central pulmonary arteries

Question 23

Complication of pulmonary HTN

Answer 23

Cor pulmonale

Question 24

Treatment of primary pulmonary HTN

Answer 24

Vasodilators

Question 25

Autoimmune disease defined by presence of anti-glomerular basement membrane antibodies

Answer 25

Goodpasture syndrome

Question 26

Typically demographic for Goodpasture syndrome

Answer 26

Young males

Question 27

Type II hypersensitivity reaction with antibodies against alveolar and glomerular basement membranes

Answer 27

Goodpasture syndrome

Question 28

Auto-antibodies to non-collagenous domain of the alpha-3 chain of collagen IV of the basement membranes

Answer 28

Goodpasture syndrome

Question 29

Young male pt presents with rapidly progressive glomerulonephritis and necrotizing hemorrhagic interstitial pneumonitis

Answer 29

Goodpasture syndrome

Question 30

Microscopy findings of the lungs in Goodpasture syndrome

Answer 30

Focal necrosis within alveolar walls with intra-alveolar hemorrhages and hemosiderin-laden macrophages. Fibrous thickening of septum.

Question 31

Immunofluorescence findings in the lungs in Goodpasture syndrome

Answer 31

Linear deposits of immunoglobulins along the basement membranes of the septal wall

Question 32

Clinical features of Goodpasture syndrome

Answer 32

Crescentic glomerulonephritis and renal failure Hemoptysis

Question 33

Causes of diffuse pulmonary hemorrhage

Answer 33

Goodpasture syndrome Idiopathic pulmonary hemosiderosis Vasculitis associated --> polyangiitis with granulomatosis

Question 34

Autoimmune disease involving upper respiratory tract and/or lung

Answer 34

Polyangiitis with granulomatosis/Wegener granulomatosis

Question 35

Diagnostic test for polyangiitis with granulomatosis

Answer 35

Transbronchial lung biopsy

Question 36

Transbronchial lung biopsy findings in polyangiitis with granulomatosis

Answer 36

Scattered, poorly formed granulomas

Question 37

Triad of polyangiitis with granulomatosis

Answer 37

Acute necrotizing granulomas of URT and/or LRT Necrotizing or granulomatous vasculitis Focal necrotizing, often crescentic, glomerulonephritis