vavular disease/vasculitis

Question 1

failure of a valve to open completely, obstructing forward flow

Answer 1

stenosis

Question 2

acquired stenosis is usually due to _____

Answer 2

chronic (recurrent) rheumatic valvular disease

Question 3

endothelial injury from a substance in cigarette smoke

Answer 3

thromboangiitis obliterans (Buerger disease)

Question 4

clinical presentation of thromboangiitis obliterans

Answer 4

cigarette smoking, < 35 years, pain of extremities, ischemic ulcers, gangrene

Question 5

pathology of thromboangiitis obliterans

Answer 5

vasculitis with thrombosis

Question 6

Longitudinal tear of the aortic media which begins in the ascending aorta and extends variable distance proximal (toward the heart) and distal to the descending aorta

Answer 6

dissecting aortic hematoma

Question 7

predisposing conditions to dissecting aortic hematoma

Answer 7

hypertension, inherited CT disorders (Marfan’s syndrom) with medial degeneration

Question 8

mitral valve stenosis is a result of _____; mostly affects ____

Answer 8

acute rheumatic fever (ARF); children (20% adults)

Question 9

clinical features of ARF

Answer 9

• arthritis (often polyarthritis)
• carditis
• erythema marginatum (skin rash)
• subcutaneous nodules

Question 10

ARF produces ____, _____, ____, _____ and other manifestations

Answer 10

myocarditis; pericarditis (fibrinous); arthralgia or arthritis; endocarditis (sterile vegetations)

Question 11

the myocarditis is characterized microscopically by ____ which are collections of _____ and _____

Answer 11

aschoff bodies; mononuclear inflammatory cells; fibroblasts (essentially granulomatous inflammation)

Question 12

recurrent bouts of ARF lead to ____ and ____ of the mitral valve and possibly other heart valves

Answer 12

severe fibrosis; calcification

Question 13

regurgitation may be caused by a variety of conditions including ____ and ____

Answer 13

IHD; infective endocarditis

Question 14

_____ is a condition in which the leaflets balloon into the left atrium during left ventricular contraction (systole)

Answer 14

mitral valve prolapse

Question 15

severe prolapse may be assoc with _____; some patients also experience ____ and ____

Answer 15

valvular regurgitation; chest pain; palpitations

Question 16

____, ______, _____, and _____ are potential complications of severe prolapse

Answer 16

endocarditis; mitral regurgitation; thromboemboli; sudden death (rare)

Question 17

in severe prolapse, also called _____, the valve cusps are large and microscopically show _____, ____, and loss of _____

Answer 17

floppy mitral valve; fragmentation; separation; collagen (myxomatous degeneration)

Question 18

aortic valve stenosis caused by ____ and ____

Answer 18

fibrosis; calcification

Question 19

aortic valve stenosis can be due to _____ or _____

Answer 19

chronic rheumatic valvular disease; advanced age (over 65 years)

Question 20

common congenital malformation which predisposes valves to fibrosis and calcification beginning at about 40 years

Answer 20

bicuspid aortic valve

Question 21

mechanisms of aortic valve regurgitation include _____, _____ and _____

Answer 21

valve cusp destruction (by endocarditis, rheumatic heart disease)
weakened valve cusps (myxomatous degeneration)
dilation of the aortic root (degeneration of the aortic media)

Question 22

infective endocarditis usually caused by a ____ in a heart valve, but may also be caused by ____ or other unusual infections

Answer 22

bacterial infection; fungus

Question 23

3 factors assoc with the pathogenesis of infective endocarditis

Answer 23

1. endocardial or endothelial injury due to abnormalities in blood flow
2. fibrin thrombi
3. organisms in the blood (sepsis)

Question 24

complications of infective endocarditis

Answer 24

1. rupture of chordae tendinae
2. spread of infection into myocardium or aorta
3. thromboembolism with infarction
4. septic thrombi with metastatic abscesses
5. valvular dysfunction and CHF

Question 25

differences in organism and tissue destruction between acute and subacute infective endocarditis

Answer 25

virulent organism in acute; organism of low virulence in subacute prominent tissue destruction in acute, less tissue destruction in subacute

Question 26

causes of vasculitis

Answer 26

``` infection (usually due to spread of adjacent infection) mechanical trauma toxins caustic substances radiation immune complexes ```

Question 27

rare before age 50; visual disturbances; claudication of jaw; polymyalgia rheumatica

Answer 27

giant cell (temporal) arteritis

Question 28

pathology of giant cell (temporal) arteritis includes _____ with giant cells and _____

Answer 28

granulomatous inflammation; fibrosis

Question 29

called "pulseless disease" due to weak pulses in the arms; usually affects young women

Answer 29

takayasu arteritis

Question 30

pathology of takayasu arteritis includes _____ inflammation with ____ involving the ____ and ____

Answer 30

granulomatous ; fibrosis; aortic arch; arch branches

Question 31

suspected that a viral infection triggers a hypersensitivity reaction; affects infants and young children

Answer 31

kawasaki disease

Question 32

microscropic polyangiitis often due to _____; involves ____

Answer 32

antigen-antibody complexes; arterioles, capillaries, and venules

Question 33

involves abnormal expression of proteinase 3 on endothelial cell surface ; involves sinuses, lungs, and kidneys (glomerulonephritis)

Answer 33

wegener granulomatosis

Question 34

acute rheumatic fever is a systemic disease, usually in children, which follows a _______ pharyngitis

Answer 34

group A beta-hemolytic streptococcal

Question 35

ARF produces:

Answer 35

- arthritis (often polyarthritis) - myocarditis/pericarditis - erythema marginatum (skin rash) - subcutaneous nodules

Question 36

predisposing factors to infective endocarditis

Answer 36

abnormal heart valves, prosthetic valves, IV drug use, intracardiac shunts, diabetes, and immunosuppression

Question 37

clinical manifestations of infective endocarditis include:

Answer 37

fever, heart murmur, fatigue, anemia, arthralgia, myalgia, splinter hemorrhages (nail bed), and roth spots (retinal hemorrhages)

Question 38

pathogenesis of immune-mediated vasculitis (noninfectious)

Answer 38

1. immune complex formation 2. anti-neutrophilic cytoplasmic antibodies (ANCAs) 3. anti endothelial cell antibodies 4. cell-mediated immunity (autoreactive T cells)

Question 39

type of ANCA with perinuclear localization ; found in microscopic polyarteritis

Answer 39

anti-myeloperoxidase (anti-MPO)

Question 40

type of ANCA with diffuse cytoplasmic distribution ; found in wegener's granulomatosis

Answer 40

anti-proteinase-3 (anti-PR3)

Question 41

anti-endothelial cell antibodies found in _____

Answer 41

kawasaki disease

Question 42

immune complex formation is a reaction to ____ or ____

Answer 42

drugs; viruses

Question 43

haphazard and segmental involvement of medium and small muscular arteries; at one time, up to 30% with disease had hep B surface antigens in serum

Answer 43

polyarteritis nodosa

Question 44

acute lesions in polyarteritis nodosa show : _____ | -healing involves progressive fibrous scarring

Answer 44

fibrinoid necrosis, thrombosis, neutrophils, aneurysms

Question 45

kawasaki disease is usually ____, but 1-2% die with _____

Answer 45

self-limited; coronary artery vasculitis

Question 46

clinical presentation of microscopic polyangiitis involves presence of ______

Answer 46

circulating anti-neutrophilic cytoplasmic antibodies (MPO-ANCA)