vavular disease/vasculitis Flashcards
failure of a valve to open completely, obstructing forward flow
stenosis
acquired stenosis is usually due to _____
chronic (recurrent) rheumatic valvular disease
endothelial injury from a substance in cigarette smoke
thromboangiitis obliterans (Buerger disease)
clinical presentation of thromboangiitis obliterans
cigarette smoking, < 35 years, pain of extremities, ischemic ulcers, gangrene
pathology of thromboangiitis obliterans
vasculitis with thrombosis
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
dissecting aortic hematoma
predisposing conditions to dissecting aortic hematoma
hypertension, inherited CT disorders (Marfan’s syndrom) with medial degeneration
mitral valve stenosis is a result of _____; mostly affects ____
acute rheumatic fever (ARF); children (20% adults)
clinical features of ARF
- arthritis (often polyarthritis)
- carditis
- erythema marginatum (skin rash)
- subcutaneous nodules
ARF produces ____, _____, ____, _____ and other manifestations
myocarditis; pericarditis (fibrinous); arthralgia or arthritis; endocarditis (sterile vegetations)
the myocarditis is characterized microscopically by ____ which are collections of _____ and _____
aschoff bodies; mononuclear inflammatory cells; fibroblasts (essentially granulomatous inflammation)
recurrent bouts of ARF lead to ____ and ____ of the mitral valve and possibly other heart valves
severe fibrosis; calcification
regurgitation may be caused by a variety of conditions including ____ and ____
IHD; infective endocarditis
_____ is a condition in which the leaflets balloon into the left atrium during left ventricular contraction (systole)
mitral valve prolapse
severe prolapse may be assoc with _____; some patients also experience ____ and ____
valvular regurgitation; chest pain; palpitations
____, ______, _____, and _____ are potential complications of severe prolapse
endocarditis; mitral regurgitation; thromboemboli; sudden death (rare)
in severe prolapse, also called _____, the valve cusps are large and microscopically show _____, ____, and loss of _____
floppy mitral valve; fragmentation; separation; collagen (myxomatous degeneration)
aortic valve stenosis caused by ____ and ____
fibrosis; calcification
aortic valve stenosis can be due to _____ or _____
chronic rheumatic valvular disease; advanced age (over 65 years)
common congenital malformation which predisposes valves to fibrosis and calcification beginning at about 40 years
bicuspid aortic valve
mechanisms of aortic valve regurgitation include _____, _____ and _____
valve cusp destruction (by endocarditis, rheumatic heart disease)
weakened valve cusps (myxomatous degeneration)
dilation of the aortic root (degeneration of the aortic media)
infective endocarditis usually caused by a ____ in a heart valve, but may also be caused by ____ or other unusual infections
bacterial infection; fungus
3 factors assoc with the pathogenesis of infective endocarditis
- endocardial or endothelial injury due to abnormalities in blood flow
- fibrin thrombi
- organisms in the blood (sepsis)
complications of infective endocarditis
- rupture of chordae tendinae
- spread of infection into myocardium or aorta
- thromboembolism with infarction
- septic thrombi with metastatic abscesses
- valvular dysfunction and CHF
differences in organism and tissue destruction between acute and subacute infective endocarditis
virulent organism in acute; organism of low virulence in subacute
prominent tissue destruction in acute, less tissue destruction in subacute
causes of vasculitis
infection (usually due to spread of adjacent infection) mechanical trauma toxins caustic substances radiation immune complexes
rare before age 50; visual disturbances; claudication of jaw; polymyalgia rheumatica
giant cell (temporal) arteritis
pathology of giant cell (temporal) arteritis includes _____ with giant cells and _____
granulomatous inflammation; fibrosis
called “pulseless disease” due to weak pulses in the arms; usually affects young women
takayasu arteritis
pathology of takayasu arteritis includes _____ inflammation with ____ involving the ____ and ____
granulomatous ; fibrosis; aortic arch; arch branches
suspected that a viral infection triggers a hypersensitivity reaction; affects infants and young children
kawasaki disease
microscropic polyangiitis often due to _____; involves ____
antigen-antibody complexes; arterioles, capillaries, and venules
involves abnormal expression of proteinase 3 on endothelial cell surface ; involves sinuses, lungs, and kidneys (glomerulonephritis)
wegener granulomatosis
acute rheumatic fever is a systemic disease, usually in children, which follows a _______ pharyngitis
group A beta-hemolytic streptococcal
ARF produces:
- arthritis (often polyarthritis)
- myocarditis/pericarditis
- erythema marginatum (skin rash)
- subcutaneous nodules
predisposing factors to infective endocarditis
abnormal heart valves, prosthetic valves, IV drug use, intracardiac shunts, diabetes, and immunosuppression
clinical manifestations of infective endocarditis include:
fever, heart murmur, fatigue, anemia, arthralgia, myalgia, splinter hemorrhages (nail bed), and roth spots (retinal hemorrhages)
pathogenesis of immune-mediated vasculitis (noninfectious)
- immune complex formation
- anti-neutrophilic cytoplasmic antibodies (ANCAs)
- anti endothelial cell antibodies
- cell-mediated immunity (autoreactive T cells)
type of ANCA with perinuclear localization ; found in microscopic polyarteritis
anti-myeloperoxidase (anti-MPO)
type of ANCA with diffuse cytoplasmic distribution ; found in wegener’s granulomatosis
anti-proteinase-3 (anti-PR3)
anti-endothelial cell antibodies found in _____
kawasaki disease
immune complex formation is a reaction to ____ or ____
drugs; viruses
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
polyarteritis nodosa
acute lesions in polyarteritis nodosa show : _____
-healing involves progressive fibrous scarring
fibrinoid necrosis, thrombosis, neutrophils, aneurysms
kawasaki disease is usually ____, but 1-2% die with _____
self-limited; coronary artery vasculitis
clinical presentation of microscopic polyangiitis involves presence of ______
circulating anti-neutrophilic cytoplasmic antibodies (MPO-ANCA)
