Lecture 21: Pathology of Vascular Disease

Question 1

PE: risk factors

Answer 1

Hypercoagulability, carcinoma/Trousseau’s syndrome, protein C/S deficiency, oral contraceptives, pregnancy

Question 2

PE: gross (massive embolism)

Answer 2

Massive embolism may show no parenychymal changes because death happened very quickly

Question 3

PE: gross (smaller embolism)

Answer 3

Wedge shaped, hemorrhagic parenchyma (due to dual blood supply), will organize (fibrous) and eventually scar

Question 4

What finding is indicative of a true embolism and not a postmortem clot?

Answer 4

Lines of Zahn

Question 5

How does the hemorrhagic infarct form?

Answer 5

Because bronchial arteries don’t supply alveoli and pulmonary artery blocked, alveoli will undergo necrosis up to bronchial blood supply, and then blood will be pumped into this space

Question 6

What happens to infarction following PE?

Answer 6

Hemorrhagic area becomes subpleural scaring (triangle shaped, retracted)

Question 7

Define pulmonary hypertension; what % of systemic pressure to reach hypertensive level?

Answer 7

Hemodynamic consequence of various conditions that lead to chronic elevation of PA pressure; at least 25% of systemic pressure (normal is 10%)

Question 8

What finding in pulmonary arteries suggests pulmonary hypertension?

Answer 8

Pulmonary atherosclerosis

Question 9

Pulmonary hypertension: primary and secondary

Answer 9

Primary = due to intrinsic abnormality of vessels; Secondary = due to something other than vessels

Question 10

When you think of primary hypertension, think of __________ lesions

Answer 10

Plexiform

Question 11

Is primary pulmonary HTN common? Who gets it? Etiology?

Answer 11

No: rare; young women; idiopathic or due to collagen vascular disease, drugs (diet drug, phenphen)

Question 12

What are some etiologies of secondary pulmonary HTN?

Answer 12

Heart disease (malformation, CHF) or severe lung disease (COPD, fibrosis)

Question 13

What is a consequence of recurrent thromboembolism?

Answer 13

Pulmonary HTN!

Question 14

How does primary pulmonary HTN present? Survival?

Answer 14

Progressive SOB, syncope, maybe sudden death; 5 years = 35%

Question 15

Grading of pulmonary hypertension: Which grade is no longer reversible?

Answer 15

Grade III: subintimal fibrosis with onion-ring appearance; marked reduplication of internal elastic membrane; arteries and arterioles resemble pipes (concentric fibrosis)

Question 16

Grading of pulmonary hypertension: Which grade is associated with plexiform lesions?

Answer 16

Grade IV to V

Question 17

Describe a plexiform lesion

Answer 17

Herniation of vessel wall –> dilated segment taking vessel wall with it and contains numerous slits like vascular channels

Question 18

What finding do you get with chronic thromboembolic disease? What do they look like?

Answer 18

Organized thrombus within arterial wall; they demonstrate recanalization

Question 19

Vasculitic disorders affect the lung: 3 more common ones

Answer 19

Granulomatosis with polyangiitis (Wegener’s granulomatosis); Churg-Strauss disease (eosinophilic granulomatosis with polyangiitis); Microscopic polyangiitis

Question 20

Describe GPA/Wegener’s: who gets it and the triad; what do you get in the lung?

Answer 20

Wide age range, mean is 50; Triad: sinus, lung, and renal involvement; multiple lung nodules

Question 21

What is positive in most cases of GPA/Wegener’s

Answer 21

C-ANCA (anti-proteinase 3)

Question 22

GPA/Wegener’s: histology

Answer 22

IRREGULAR, parenchymal necrosis w/ neutrophils surrounded by scattered giant cells/histiocytes

Question 23

Why don’t you get well-formed granulomas in GPA/Wegener

Answer 23

Necrosis FIRST due to autoimmune disorder with giant cells reacting SECOND (unlike reaction to an antigen FIRST)

Question 24

GPA/Wegener: vasculitis component

Answer 24

Medium sized arteries/veins –> capillaries

Question 25

GPA/Wegener: earliest manifestation

Answer 25

Palisaded microgranulomas

Question 26

Churg-Strauss: triad

Answer 26

Asthma, eosinophilia, vasculitis

Question 27

What separates Churg-Strauss from Wegener’s

Answer 27

Neuropathy and cardiac involvement (but NOT renal); EOSINOPHILIC disease

Question 28

Churg-Strauss: radiograph

Answer 28

Multifocal infiltrates that may change over time

Question 29

Churg-Strauss: histology

Answer 29

Histology – asthmatic bronchitis, eosinophilic pneumonia, STELLATE GRANULOMAS and vasculitis

Question 30

Churg-Strauss: granulomas

Answer 30

Palisaded histiocytes and multinucleated giant cells, surrounding a central necrotic zone replete with eosinophils

Question 31

Microscopic polyangiitis “ANCA”

Answer 31

P-ANCA

Question 32

Microscopic polyangiitis (MPA): describe

Answer 32

Immune vasculitis restricted to arterioles, venules, and capillaries with common kidney involvement

Question 33

MPA on biopsy: what you see what what you don’t

Answer 33

Diffuse alveolar hemorrhage with neutrophilic capillaritis; No granulomas, giant cells, eosinophilia

Question 34

MPA: where do you find neutrophils?

Answer 34

Within septa (not in the airspaces themselves)

Question 35

When someone has ongoing (> a day or so) hemorrhage in the alveoli, what happens next?

Answer 35

Hemosiderin deposition in the tissue and within the macrophages

Question 36

Diffuse alveolar hemorrhage: common? dangerous? clinical symptom? typical association?

Answer 36

Rare but life-threatening (respiratory failure), you will see hemoptysis; typically associated with microscopic polyangiitis