102014 peripheral vasc dis

Question 1

aneurysm

Answer 1

localized dilatation of blood vessel or heart

Question 2

true aneurysm

Answer 2

involves all three layers of wall

Question 3

false aneurysm

Answer 3

wall defect leading to extravascular hematoma

Question 4

arterial dissection

Answer 4

blood enter the wall of the artery

Question 5

in the setting of atherosclerosis/inflam, what can predispose to aneurysm formation?

Answer 5

polymorphisms of matrix metalloproteinase or tissue inhibitors of metalloproteinase genes

also increased MMP activity

Question 6

pathogenesis of aneurysms

Answer 6

factors affecting collagen structure or fxn

loss of smooth muscle cells (due to atherosclerosis leading to thickening of intima and ischemia of inner media. or due to systemic HTN narrowing the vasa vasorum leading to ischemia of OUTER media)

Question 7

common causes of anuerysms

Answer 7

atheroslcerosis –abodominal aorta

HTN – ascending aorta

Question 8

uncommon causes of aneurysms

Answer 8

congenital defects
infections (bacteria, fungi) -mycotic, syphilis
trauma
vasculitis
genetic defects in collagen

Question 9

mycotic anueryms

Answer 9

septic emboli, direct extension, direct infec by circulating organisms

Question 10

what is the most common location of aortic aneurysm

Answer 10

abdominal aorta-below renal arteries and above bifrucation

Question 11

aortic aneuryms are more common in whom

Answer 11

men, smokers

most common cause is atherosclerosis

Question 12

morphology of aortic aneurysm

Answer 12

thinning and destruc of media
mural thrombus
saccular or fusiform
variants are: inflammatory (unknown cause), mycotic (secondary infec of an atherosclerotic wall)

Question 13

aggressive managment is done for AAA greater than

Answer 13

5 cm, as the risk of rupture is proportional to size

Question 14

thoracic aortic aneurysms causes

Answer 14

HTN (most common)
Marfan’s syndrome
syphilis

Question 15

aortic dissection

Answer 15

blood btwn and along laminar planes of media

causes a blood filled channel that easily ruptures

Question 16

pathogenesis and etiology of aortic dissec

Answer 16

HTN in over 90% of cases
connective tissue abnormality in association with Marfan’s syndrome or Ehlers Danlos

ascending aorta is most commonly involved

Question 17

morphology of aortic dissec

Answer 17

intimal tear within 10 cm of aortic valve
dissection plane btwn middle and outer thirds of wall in media

in HTN pts: vasa vasorum has hyaline arteriolosclerosis and media has loss of sm musc cells.

Question 18

cause of intimal tear in aortic dissec?

Answer 18

not usually known

Question 19

clinical course of aortic dissec

Answer 19

dependent on level of aorta involved

sharp pain of anterior chest
uneven pulses and widened mediastinum

Question 20

classic symptoms of aortic dissec

Answer 20

sudden onset of tearing or stabbing pain in anterior chest radiating to back

Question 21

pathogenesis of vasculitis

Answer 21

immune mediated processes
infectious
unknown

Question 22

pathogenesis of immune mediated vasculitis

Answer 22

immune complex mediated-SLE, hypersensitivity to drugs, viral infec like Hep B

ANCA associated

other mechanisms (antibodies to endothelial cells-Kawasaki’s)

Question 23

ex of vasculitis of infectious origin

Answer 23

direct invasion:
classic ex is syphilis
Aspergillus and mucormycosis

indirect:
immune mechanisms triggering cross-reactivity

Question 24

vasculitides of unknown origin

Answer 24

giant cell arteritis
Takayasu
PAN

Question 25

what is the most common systemic vasculitis in adults

Answer 25

giant cell temporal arteritis

Question 26

giant cell (temporal) arteritis

Answer 26

affects aorta and major branches, especially temporal artery, opthalmic, vertebral arteries

Question 27

clinical presentation of giant cell arteritis

Answer 27

greater than 50 years old often painful superficial temporal artery, diplopia, visual loss, headache increased ESR

Question 28

gross morphology of giant cell arteritis

Answer 28

nodular thickenings of artery with narrowed lumen patchy (discontinuous) segments affected

Question 29

histology of temporal arteritis

Answer 29

granulomatous inflam of inner half of media around internal elastic lamina

Question 30

takayasu arteritis

Answer 30

``` pulseless disease (upper extremities) ocular disturbances ``` Japanese women younger than 40 granulomatou inflam of AORTIC ARCH AND ITS BRANCHES pulmonary arteries are involved in 50%. coronary and renal arteries may be involved

Question 31

histology of Takayasu arteritis

Answer 31

lymphocytes giant cells collagenous fibrosis

Question 32

PAN

Answer 32

systemic SEGMENTAL, TRANSMURAL, NECROTIZING inflam of small or medium sized muscular arteries renal and visceral arteries affected SPARES LUNGS young adults (30% with Hep B antigen)

Question 33

classic presentation of PAN

Answer 33

rapidly accelerating HTN (renal artery involvement) abdominal pain and bloody stools peripheral neuritis

Question 34

Kawasaki syndrome

Answer 34

large, medium and small arteries OFTEN CORONARIES with aneurysm formation mucocutaneous lymph node syndrome (mucous membrane inflam, enlarged lymph nodes) 80% under 4 years

Question 35

histology of Kawasaki's

Answer 35

necrosis and inflam aneurysms resembles PAN

Question 36

MPA

Answer 36

arterioles, capillaries, venules MPO-ANCA in 70%

Question 37

Wegener's granulomatosis

Answer 37

necrotizing vasculitis GRANULOMAS of lung and or upper resp tract glomerulonephritis PR3-ANCAs present in more than 95% males more than females

Question 38

Churg Strauss syndrome

Answer 38

allergic granulomatosis with angiitis small vessel vasculitis associated with asthma, allergic rhinitis, lung infiltrates, infiltration of vessels by EOSINOPHILS, EXTRAvascular necrotizing granulomas MPO-ANCA is present in minority

Question 39

clinical symptoms of Churg Strauss

Answer 39

palpable purpura GI bleed renal impairement cardiomyopathy

Question 40

Thromboangiitis obliterans/Buerger disease

Answer 40

inflam and thrombosis of medium to small sized muscular arteries and secondarily the veins and nerves tibial and radial arteries smokers Israaeli, Indian, Japanese painful ischemic disease gangrene of limbs, requires amputation

Question 41

Raynaud phenomenon

Answer 41

paroxysmal pallor or cyanosis of fingers, toes, nose, ears primary-recurrent vasospasm of unknown case (exaggerated response to cold) secondary-arterial insufficiency due to narrowing and can be due to SLE, systemic sclerosis, atheroslcerosis, Buerger disease