Term
why is the endothelium key in atherosclerosis? |
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Definition
it has pro and anti thrombotic capabilities - however when injured, its prothrombotic capabilities take over |
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Term
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Definition
atherosclerosis - a major cause of morbidity/mortality in western countries |
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Term
what is the difference between arteriosclerosis and atherosclerosis? |
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Definition
arteriosclerosis is a more generalized term that doesn't pertain to a specific morphology |
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Term
what is monckeberg's arteriosclerosis? |
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Definition
calcified arteriosclerosis that is not usually a danger b/c a patent lumen is preserved. usually seen on x rays, often post mortem. it may falsely elevate BP |
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Term
what is arteriolosclerosis? |
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Definition
hardening of the arterioles |
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Term
what are the risk factors for atherosclerosis? |
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Definition
age, sex, familial predisposition, hyperlipidemia, HTN, smoking, diabetes, homocystine (associated w/stroke, CAD, PVD), and lipoprotein (a) - altered form of LDL associated with coronary artery and cerebrovascular risk independent of total cholesterol or LDL levels |
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Term
what is c-reactive protein important for? |
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Definition
c-reactive protein is a marker for inflammation, is is an acute phase reactant synthesized in the liver which can activated endothelial cells and induce a prothrombotic state. it also increases adhesiveness of endothelium for leukocytes and strongly predicts the risk of MI, stroke, PVD, and sudden death (*link between inflammation and atherosclerosis*) |
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Term
how do accumulating risk factors for atherosclerosis interact? |
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Definition
risk factors for atherosclerosis have a cumulative effect |
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Term
what do vessels look like in malignant HTN? benign? |
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Definition
malignant: onion-skinning, narrowing. benign: glassy, hyaline |
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Term
what makes up the morphology of an atherosclerotic plaque? |
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Definition
smooth muscle proliferates, engulfs lipids, and deposits collagen |
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Term
how are LDLs oxidized in atherosclerotic plaques? |
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Definition
by ROSes released by macrophages |
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Term
what are one of the key inflammatory cells in atheroma formation? |
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Definition
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Term
how can smooth muscle cells help keep atherosclerotic plaques stable? |
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Definition
by producing ECM molecules |
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Term
where is the most common area of atherosclerotic plaque deposition? what are the next most common places in descending order? |
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Definition
the abdominal aorta, followed by the coronary arteries, thoracic aorta, carotid arteries, and circle of willis |
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Term
what can cause a plaque to become unstable? |
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Definition
cell death/degeneration, inflammation, plaque growth, remodeling of plaque/wall ECM, organization of thrombus and calcification |
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Term
what is a major risk factor in development of atherosclerotic plaques macro and microvascularly? |
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Definition
diabetes, macrovascularly: large/medium sized arteries involved leading to increased atherosclerosis/increased MIs, increased strokes, and increased gangrene. microvascularly: retina, kidney, and peripheral nerves are affected. three metabolic pathways are involved: formation of advanced glycation end products, activation of protein kinase C, and disturbance in polyol pathways |
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Term
what are AGEs in diabetes? |
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Definition
advanced glycation end products - formed from the reaction between intracellular glucose derived dicarbonyl percursors w/amino group of intracellular and extracellular proteins |
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Term
what can increased activation of protein kinase C lead to? |
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Definition
increased production of VEGF -> which can lead to proliferative retinopathy as well as increased activity of vasoconstrictor endothelin 1 and decreased activity of vascoconstrictor endothelin nitric oxide synthase |
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Term
what causes 10% of vasculitis? |
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Definition
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Term
can vasculitis be associated with viral infections? |
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Definition
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Term
what are the diseases causing vasculitis associated with anti-endothelial antibodies? |
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Definition
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Term
what is the difference between c-ANCA and p-ANCA? |
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Definition
c-ANCA: cytoplasmic target - proteinase 3, p-ANCA: perinuclear target - myeloperoxidase (MPO) |
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Term
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Definition
polyarteritis nodosa is seen in small -> medium sized arteries, (not arterioles, capillaries and venules). renal and GI involvement is common and pulmonary vessels are usually spared |
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Term
who is PAN usually seen in? how do these pts present? |
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Definition
generally young adults who may have acute/subacute/chronic lesions along with HTN/wt loss/fatigue/melana |
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Term
what is often the cause of death in PAN? |
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Definition
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Term
what is the morphology of PAN lesions? |
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Definition
PAN lesions are segmental, transmural and have necrotizing inflammation. they are characterized by fibrinoid necrosis, fibrous thickening of the vessel wall and all stages can co-exist in different or the same vessels |
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Term
can PAN cause glomerulonephritis? |
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Definition
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Term
how many PAN cases are positive for the Hep B antigen? |
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Definition
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Term
can PAN be lethal? what will bloodwork for it show? |
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Definition
yes, if untreated. PAN can cause elevated ESR and leukocytosis |
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Term
is PAN associated with c-ANCA? |
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Definition
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Term
are lesions in microscopic polyangitis all around the same age? |
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Definition
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Term
what blood vessels does microscopic polyangitis affect? |
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Definition
arteries, capillaries and venules (different than PAN) |
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Term
can a skin bx be dx for microscopic polyangitis? |
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Definition
yes b/c it can present as palpable purpura of the skin, mucous membranes, lungs, brain, heart, GIT, kidney, and muscle |
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Term
are glomerulonephritis and pulmonary capillarities associated with microscopic polyangitis? |
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Definition
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Term
is p-ANCA associated with microscopic polyangitis? |
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Definition
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Term
do you see granulomatous inflammation with microscopic polyangitis? |
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Definition
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Term
how do neutrophils appear in microscopic polyangitis? |
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Definition
neutrophils are fragmented in microscopic polyangitis |
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Term
what are presentations of microscopic polyangitis? |
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Definition
hemoptysis, arthralgia, abdominal pain, hematuria, proteinuria, and hemorrhage |
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Term
what is churg strauss associated with in terms of pt presentation? |
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Definition
allergic rhinitis, broncial asthma and eosinophilia - due to hyperresponsiveness of allergic stimulus |
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Term
is p-ANCA associated with churg-strauss pts? |
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Definition
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Term
is vasculitis in churg strauss granulomatous? |
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Definition
yes with neutrophilic necrosis |
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Term
how does churg strauss appear histologically? |
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Definition
as infiltration of vessels and perivascular tissue by neutrophils |
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Term
what are the primary causes of morbidity and mortality in churg strauss? |
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Definition
coronary arteritis and myocarditis |
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Term
what is giant cell arteritis also known as? where does it affect? |
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Definition
temporal arteritis - which is granulomatous inflammation and fragmentation of the internal elastic membrane in large to small arteries in the temporal/opthalmic/vertebral and aortic arteries |
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Term
can giant cell cause blindness? |
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Definition
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Term
what is takayasu arteritis? where are symptoms often seen? |
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Definition
granulomataus vasculitis of medium to larger arteries. ocular symptoms are common along with weakening of upper extremity pulses. |
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Term
what arteries are usually affected by takayasu arteritis? |
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Definition
the aorta and aortic arch (thickening) as well as possibly the pulmonary artery |
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Term
what are the HLA associations for takayasu arteritis? |
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Definition
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Term
how does takayasu arteritis appear histologically? clinically? |
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Definition
histologically: giant cells and patchy necrosis of the media. clinically: blindness, coldness, numbing of the extremities, HTN, and neurologic deficits. arteries are subject to concentric narrowing |
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Term
what can cause mycotic aneurysms? |
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Definition
infectious arteritis (bacterial pneumonia and TB) |
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Term
what might be seen in infectios arteritis histologically? |
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Definition
infiltration of neutrophils |
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Term
what is kawasaki disease also known as? what does it present with? |
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Definition
mucocutaneous lymph node syndrome - and can present as fever, conjunctivitis, oral erythema and erosion |
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Term
what is the mnemonic for kawasaki disease? |
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Definition
Kids, Koronary, Konjunctivitis |
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Term
what other form of vasculitis does kawasaki disease appear similar to histologically? |
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Definition
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Term
what kind of vasculitis is wegners? |
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Definition
granulomatous, necrotizing |
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Term
can wegners affect the kidneys? |
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Definition
yes with focal necrotizing glomerulonephritis |
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Term
is there ANCA involvement with wegener's? |
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Definition
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Term
what is thromboangiitis obliterans? what size arteries does it affect? who in the pt population is most affected by it? |
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Definition
a hypersensitivity to intradermally injected tobacco extracts - also called buerger's disease. it affects medium sized arteries - appearing as sharply segmented acute and chronic vasculitis. it is seen mostly in male smokers <35 yrs old |
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Term
what are cavernous hemangiomas associated with? |
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Definition
von hippel-lindau disease |
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Term
what are common causes for angiosarcomas? |
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Definition
VAT - vinyl chloride, arsenic, and thorotrast |
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Term
what is the genetic component of von hippel lindau disease? |
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Definition
deletion of VHL gene (tumor suppressor) on chr 3 (autosomal dominant) |
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