SFP: vascular lung disease

Question 1

What three diseases are associated with alveolar hemorrhage?

Answer 1

Goodpasture syndrome, idiopathic pulmonary hemosiderosis, and granulomatosis with polyangiitis

Question 2

What is the triad associated with alveolar hemorrhage syndromes?

Answer 2

Hemoptysis, anemia, and diffuse pulmonary infiltrate on imaging

Question 3

Describe Goodpasture syndrome generally.

Answer 3

Antibodies to basement membranes cause dysfunction of vessels of lungs and kidneys that causes bleeding

Question 4

What causes hemorrhagic interstitial pneumonitis?

Answer 4

Goodpasture syndrome

Question 5

Goodpasture syndrome may present with… (3)

Answer 5

Proteinuria, hematuria, acute renal failure

Question 6

Describe what is seen in the lungs with Goodpasture syndrome.

Answer 6

Hemorrhage in the alveoli, inflamed capillaries, hypertrophy, and possible hemosiderin

Question 7

What is a signature pattern seen in testing of Goodpasture syndrome?

Answer 7

Linear immunofluorescence of immunoglobulin

Question 8

Describe idiopathic pulmonary hemosiderosis.

Answer 8

A rare condition usually in kids and infants that causes bleeding in the alveolar spaces without presence of renal disease, antibodies to basement membrane, and variable clinical course and response to steroids. Lots of hemosiderin-laden macrophages in alveolar spaces

Question 9

What is the triad seen with granulomatosis with polyangiitis?

Answer 9

Necrotizing granulomas, pulmonary vasculitis, and crescentic glomerulonephritis

Question 10

What is seen on x-ray with granulomatosis with polyangiitis?

Answer 10

White infarcts due to necrotizing vasculitis of medium sized arteries

Question 11

What is the typical patient population of granulomatosis with polyangiitis?

Answer 11

50-year-old men with sinusitis, pneumonitis, and renal disease

Question 12

c-ANCA is seen in…

Answer 12

granulomatosis with polyangiitis

Question 13

What is the cornerstone treatment of granulomatosis with polyangiitis?

Answer 13

steroids and immunosuppressants

Question 14

What is an issue with granulomatosis with polyangiitis?

Answer 14

opportunistic infection and renal failure

Question 15

What do we see in the lungs with granulomatosis with polyangiitis?

Answer 15

necrotizing vasculitis, parenchymal granulomas, nodules

Question 16

___ impacts capillaries while ___ can impact other/larger vessels.

Answer 16

Goodpasture’s, granulomatosis with polyangiitis

Question 17

What are predisposing factors to pulmonary thromboembolism?

Answer 17

Trauma, stasis, surgery, contraceptive pills

Question 18

Describe clinical outcomes of pulmonary embolism.

Answer 18

Many are small and silent and are eventually lysed. Large ones may be instantly fatal, or some may cause infarction. People can also have recurrent PEs

Question 19

What can recurrent PEs cause?

Answer 19

Pulmonary hypertension and chronic right heart strain

Question 20

What is a main factor that may cause varied presentation of pulmonary infarction?

Answer 20

Dual blood supply to the lung (pulmonary and bronchial arteries)

Question 21

What infarct shape is associated with pulmonary embolism?

Answer 21

Wedge-shaped

Question 22

What are some treatments of PE?

Answer 22

Anticoagulant, thrombolysis, surgery

Question 23

What are the qualifications for pulmonary hypertension?

Answer 23

Mean pulmonary artery pressure greater than 25 at rest or 30 with exercise

Question 24

Is primary or secondary pulmonary hypertension more common?

Answer 24

Secondary

Question 25

Describe primary pulmonary hypertension.

Answer 25

Pulmonary hypertension with no other identifiable cause. It is rare and is often in women of reproductive age

Question 26

What pathophysiologic mechanisms can cause secondary pulmonary hypertension?

Answer 26

1. Increased pulmonary blood flow (VSD, PDA)
2. Prolonged left atrial hypertension (LV failure)
3. Vascular obstruction (PE, stenosis, pulmonary veno-occlusive disease)
4. Pulmonary vasoconstriction (COPD, sleep apnea, kyphoscoliosis)

Question 27

People with sickle cell are prone to…

Answer 27

Pulmonary hypertension via vascular obstruction

Question 28

What are plexiform lesions?

Answer 28

Lesions formed only in primary hypertension