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109 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Evolution of arterial wall changes in the response to injury hypothesis. 1, Normal. 2, Endothelial injury with adhesion of monocytes and platelets (the latter to sites where endothelium has been lost). 3, Migration of monocytes and smooth muscle cells into the intima. 4, Smooth muscle cell proliferation in the intima with ECM production. 5, Well-developed plaque
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describe this pathology
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hyaline arteriolosclerosis: pink homogenous hyaline thickening of arteriolar walls
what conditions is this seen in? |
older individuals
generalized HTN diabetes major component of benign nephrosclerosis |
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describ the pathology
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hyperplastic arteriolosclerosis: onion skin concentric laminated thickening of vessel walls
what is this seen in? |
acute elevations or severe HTN -->"malignant HTN"
vasculitides hypoxemia of pregnancy |
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list the 5 consequences of entothelial injury
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- Increase permeabillity of vessel wall
- Promote PLT adherence -Promote monocyte/macrophage adherence/entry -Alter vasomotor tone - Stimulate SMC |
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describe how monocytes are involved in the formation of atherosclerosis, list 5 steps
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-adhere to altered endothelium
-enter intima -engulf LDL cholesterol (scavenger pathway) -oxidize LDL -Secrete products (IL-1) to recruit more inflammatory cells |
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Once lipoproteins (LDL/VLDL) and lipids have gained entry into vessel wall and are modified by oxidation, what are the three things that OX-LDL does?
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1. it is cytotoxic to endothelial cells and smooth muscle cells
2. cehmotactic for monocytes, increases monocytes adhesion, inhibits macrophage mobility |
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How to SMCs contribute to atheroslerosis
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1. they become foam cells = become phagocytic
2. they take up lipids 3. they profliferate in the intima 4. make growth factors : PDGF 5. they make collagen, elastin, glycoproteins |
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what are the consequeneces of atherosclerosis
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1. narrowed vessels lumen: limited blood flow (no symptoms), reversible ischemia wihth increased demand (claudation walking 10 blocks, angina after exercize)
thrombosis: vessel occluded, sudden severe ischemia, infarct generate emboli: thrombosis at distant site weakened wall --> aneurysm |
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what is the definition of an aneurysm
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abnormal localized dilation of blood vessel
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differentiate between a true aneurysm and a false aneurysm
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a true aneurysm is bound by all 3 arterial walls components, where as a false aneurysm is a breach in the vessle wall leading to a hematoma that communicates with the intravascular space
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what are the two most important causes of aneurysms?
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atherosclerosis and cystic medial degeneration
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what are some other causes (notes list 4)
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infection (mycotic aneurysm), vasculitis, trauma, congenital defects
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List 3 true aneurysms and 1 that can be both
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Atherosclerosis
syphilis congential (berry) MI if the ventricular wall is intact |
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List a false aneurysm and 1 that can be both
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vascular graft leak,
also MI if the ventricular wall ruptures |
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what is cystic medial degeneration
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- elastic fragmentation and clefts filled with ECM (not truly cysts)
**Inflammation and necrosis are ABSENT -accompanies Marphan Syndrome |
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what infection leads to a mycotic aneurysm
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salmonella gastroenteritis
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notice the true aneurysm on the left with all 3 layers buldgin out. On the right, in the false aneurysm, only the adventitia is buldging out, the break is in the media and intima
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If breaks back in to the lumen = double barrel
If breaks out of adventitia = rupture |
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Why wouldn't you have an abdominal aortic aneurysm fixed as soon as you knew about it?
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High operative mortality --> 5% for unruptured and >50% if ruptured
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What is the most common location for aneurysm?
Why? |
Between the renal arteries and aortic bifurcation -->according to Goljan it is because there is no vaso vasorum below the renal artery, and makes it more susceptible to ischemic damage and atherosclerotic build up
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What is a common co-existing problem? why?
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ischemic heart dz -->because both processes are secondary to atherosclerosis
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% risk of rupture of a given aneurysms in the size: <4, 4-4.9. 5 - 5.9, >6
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Robbins:
<4=0% 4-4.9 = 1% 5-5.9 = 11% >6 = 25% Notes: <5 - minimal risk >5 - 5-10% risk >6 - 25% risk |
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what demographic does abdominal aortic aneurysm typically occur in?
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men > 50
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what causes thoracicc AA?
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syphillis (causes obliterative endarteritis of vasa vasorum that results in ischemia of media and loss of elastic tissue
giant cell arteritis |
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how is syphillitic "tree-barking" caused?
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contraction of fibrous scars that wrinkles the intervening uninvolved intima
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what are 2 other syphillitic aortitis complications
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1. atheromatous involvment of aortic root (may occlude coronary ostia)
2. aortic ring dilation leading to aortic valve insufficiency and massive LV hypertrophy called cor bovinum or cows heart |
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what condidtions is aortic dissction seen
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marfans syndrome
HTN iatrogenic pregnancy (Rarely see in atherosclerosis or syphillis) |
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what is the most common cause of dissection
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cystic medial degeneration
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what isthe genetic defect cause of Marfans
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AD defect in fibrillin
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what are the two types of aneurysms
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A - ascending aorta or entire aorta
B- below subclavian |
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what are the symptoms of syphillitic aneurysm
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heart failure, impigement of surrounding thoracic organs, rarely rupture
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what is the pathogenesis of syphillitic aneurysms
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-Arise in tertiary syphillis
- inflammation of vasa vasorum with eventual obliterative endarteritis, **plasma cells common |
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name the pathology here and the underlying dz
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"tree barking" due to syphillis -->which leads to ischemic injury and and scarring of the aorta
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define aortic dissection
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dissection of blood along laminar planes of aortic media forming a blood filled intramural channel -
- rupture = massive hemorrhages +/- sudden death |
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What are the 2 groups that aortic dissection typically occurs in?
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1. Men 40-60 who are hypertensive - nonspecific degenerative histologic changes
2. People with connective tissue diseases affecting the aorta (Marfan) |
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Describe Marfan's syndrome
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marked elastic tissue fragmentation and disruption with cystic/clefted spaces containing amorphous extracellular matrix = CYSTIC MEDIAL DEGENERATION -->defect in fibrillin gene
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describe the pathology on the which side? compare to the normal
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the pathology shown is of marfans with cystic medial degeneration, a marked elastin fragmentation, that form areas devoid of elastin that resembles cystic spaces
the right is normal - showing layered pattern of elastic matrix |
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what is the clinical presentation of aortic dissection
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sudden onset of excruciating pain starting int he anterior chest and radiating to the back -->often described as "RIpping" or "Tearing"
- pain moves down as dissection descends |
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KNOW KNOW KNOW
what is the most common form of vasculitis seen in adults? |
Temporal arteritis
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what is the most common form of vasculitis seen in children
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Henoch- Schenlein purpura
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Define Vasculitis
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Inflammation of the vessel wall / vessel destruction
-can affect large and small vessles -wide spectrum of clincial manifestations -can be a direct infection/immunologic/unknown |
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in the pauci-immune vasculitides, what is the target antigen for Cytoplasmic or cANCA
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PR3 - (protinase) a neutrophilic granule
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in the pauci-immune vasculitides, what is the target antigen for Perinuclear or pANCA
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MPO (myeloperoxidase)
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what is typically found in (ancas) in wegener's granulomatosis?
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cANCA (95%)
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what is commonly found in microscopic polyangitis?
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pANCA
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what is typically found in Churg Strauss
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pANCA
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How are ANCAs used clinically
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quantitative diagnostic markers - the levels reflect the degree of inflammatory activity and recurrence
can be used to monitor therapy as well |
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what vasculitides affect large vessels, including the aorta, large branches to the extremeties, head and neck
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giant cell (temporal) arteritis
takayatsu arteritis |
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what vasculitides affect medium vessels, including the main visceral arteries and their branches
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polyarteritis nodosa
kawaski disease |
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which vasculitides affect small vessels, including arterioles, venules, capillaries, and occasionally small arteries
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Wegener granulomatosis
Churg-Strauss Syndrome Microscopic polyangitis |
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Figure 11-23 Giant-cell (temporal) arteritis. A, H&E stain of section of temporal artery showing giant cells at the degenerated internal elastic lamina in active arteritis (arrow). B, Elastic tissue stain demonstrating focal destruction of internal elastic lamina (arrow) and intimal thickening (IT) characteristic of long-standing or healed arteritis. C, Examination of the temporal artery of a patient with giant-cell arteritis shows a thickened, nodular, and tender segment of a vessel on the surface of head (arrow). (C from Salvarani C et al.: Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 347:261, 2002.)
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which large vessel vasculitide is associated with polymyalgia rheumatica?
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giant cell temporal arteritis
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which large vessel vasculitide occuris in patients under 50?
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Takayasu
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Which medium vessel vasculitide is associated with mucocutaneous lymph node syndrome?
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kawaski
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what is mucocutaneous lymph node syndrome
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acute self limited syndrom with fever, conjunctival and oral erythema, desquamating skin rash, cervical lymph node enlargement
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which small vessel vasculitide contains eosinophil-rich granulomas of respiratory tract
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Churg strauss syndrome
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what 2 other features may be found in churg strauss?
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asthma and circulating eosinophilia
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Necrotizing glomerulonephritis is common in which two small vessel vasculitides?
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Wegener and microscopic polyarteritis
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which is the "pulseless disease"
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Takayasu (weakening of the upper extremity pulses due to intimal thickening)
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which large vessel vasculitide affects the aortic arch?
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takayasu
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which vessel vasculitis is assocated with Hepatitis B in 30% of cases?
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Polyarteritis Nodosa
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What is the leading cause of heart disease in kids/
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kawasaki (20% develop cardiac sequelae)
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how is kawasaki treated
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aspirin and IV gamma globulin
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which has lesions of the same age that affect vessels?
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microscopic polyangiitis/leukocytoclastic vasculitis
-in contrast to polyarteritis nodosa, where different stages of lesions may be present in the same vessels or other vessels |
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what HLA is associated with Beurger's disease?
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Buerger(thomboangiitis obliterans) is associated with HLA-A9 and HLA-B5
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What is rare about the histologic inflammatory process associated with Buerger dz
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the thrombus may contain microabcesses and the inflammation may extend through the vessel wall to involve veins and nerves
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what infectious agent invades vessels
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fungus in diabetics - aspergillus and mucor
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how are lymphangiomas distinguished from hemangiomas?
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by the absnse of blood in the spaes (may also see lymphatic valves)
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what is the painful tumor of the digits called
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glomus tumor - excision is curative
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with what infectious agent is bacillary antiomatosis associated?
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Bartonella henselae (causes cat scratch fever in immunocompetent people, cat flea vector)
b quintonna (causes trench fever in WWI soldiers, louse vector) Affects immunocompromised HIV pts |
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with what infectious agent is Kaposi's sarcoma associated
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HHV-8
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General Vasculitis facts:
-requires evidence of what? -type of inflamation? -classification based on? |
Must have evidence of vessle wall damage (ie thrombi, granuloma)
- inflammation can be granulomatous -classification based on **size of vessel (hist & loc), pathogenesis, and clinical presentation |
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what types of infection lead to vasculitis
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viral, fungal, bacterial, ricketsial, spirochetal
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What non-infectious causes of vasculitis
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-**most important is Immunologic injury -->more likely to have systemic complications
immune complex (HSP, SLE), Direct Ab Attack (goodpastures), ANCA assoc, cell mediated (transplant) |
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what type of vasculitis is ANCA (antineutrophil cytoplasmic antibodies) helpful with? what does it correlate with? how is it demonstrated
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it is demonstrated by immunoflorescent assays
- very helpful in patients with small vessel vasculitis - correlates with active disease and disease severity |
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what type of inflammation is seen in both large vessel vasculitides
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granulomatous
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what are the main differences between temporal and takayasu's
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temporal is in older people affecting the temp artery, with flu like prodrome and point tenderness, and blindness, increases Sed Rate
where takayasu is in youger patients, it affects the aortic arch and major branches, is called "pulseless disease" (UE only, LE fine), can have visual defects, |
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describe pathology
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takayasu - in upper left see giant cells, mediated by lymphocytes, has a prediliction for women, and japanese
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describe the pathology
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PAN - this is segmental necrosis, (note the fibrin in the bottom left of the lumen, but relatively normal lumen at the top right. ) with thrombotic occlusion of lumen of sm artery
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describe the pathology
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PAN - small aneursyms at vessel bifurcation are common
-endothelium is gone or replaced by thrombus, -in the midddle pciture note hte dense inflamatory cells, with no muscles cells -systemic symptoms |
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with PAN what demographic is typically involved?
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all ages, often younge adults -->presents with fever malaise and weight loss
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List the organs PAN typically does and does NOT affect
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PAN can affect the GI, muslces, other systems,
NOT THE LUNG can affect KINDEYS --.NO GLOMERULONEPHRITIS |
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List the important features of PAN
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can involve smaller vessels, but not arterioles
SEGEMENTAL disease involves sharply demarcated portions of affected vessels that often have secondary thrombosis leading to distal ischemic injruy |
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PAN important association
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HEPATITS B
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what is an acute illness in children and infants, presents with fever, lyphadenopathy, skin rash, oral/conjunctivital erythema, and affects CORONARY ATERITIS (20%)
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Kawasaki - legions histo sim to PAN
tx with Immunoglobulins NOT steroids coronary arteritis important becuase it kills people |
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List the immune complex smal vessel vasculitides
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Henoch-Schlonlein purpura
immune complex GN cryoglobulinemia graft rejection SLE/RA |
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Describe the presentation of Microscopic Polyangiitis (polyarteritis, leukocytoclastic vasculitis)
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-ALL lesions at same stage
-INVOLVES KIDNEYS & LUNGS -palpabel purpuras -**P-ANCA - neutrophils in whole and frag forms inflitrating vessel walls and causing fibrinoid damage |
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Churg-Strauss
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necrotizing vasculitis + granulomas
EOSINOPHILS -->strong association with allerigic rhinitis and bronchial asthma, often persipheral eosinophilia p-ANCA in Half |
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What is wegener's triad
typically found in 40 yo men, c-ANCA in majority |
necrotizing granulomatous vasculitis,
necrotizing granulomas of URT, LRT, & both renal dz -->with glomerulonephritis |
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Henoch-Schonlein purpura characterized by purpuric skin lesions, what does it present with and what are other clinical findings?
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Presents with abdominal pain
IgA dominant immune complex deposits in capillaries, venules, and arterioles See palpable purpuras on buttocks and lower extremities, hematuria, polyarthritis |
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what type of vessel does buerger dz affect and what type inflammation is it?
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it affects the small/medium arteries and veins.
it is segemental thrombosing acute and chronic inflammation, that is VERY PAINFUL |
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who does buergers dz primarily affect
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younge male smokers
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Buergers may lead to gangrene, what are the tx
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quit smoking, may slow progression. Otherwise can progress to amputation
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what is the difference between raynaud disease and phenomenon
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Disease is paroxsymal pallor/cyanosis of finger tips and toes, that is secondary to vasospasm of the small arteries and arterioles. It is common in younge women as a exaggerated vasomotor response to cold/stress
Whereas, phenomenon is extremity arterial insufficiency secondary to arterial narrowing dute to other conditions/dz GIVE red-white-blue |
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what are the two venous diseases
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varicose veins and thrombophlebitis
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what is the common clinical finding of varicose veins
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ulcers and stasis dermatitis often develop
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what causes varicose veins
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they are abnormally dilated tortous veins esp in LE, secondary to chronically elevated pressures, valves become incompetent.
-luminal thromosis can occur but emboli are rare |
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What is thrombophlebitis
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venous inflammation
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what does DVT lead to
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Pulmonary Emboli
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What is Trousseau Syndrome
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multiple venous thrombi in the setting of malignacy (espcially adenocarcinoma)
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What are the four types of hemangiomas
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Capillary
Juvenile capillary (strawberry) cavernous Pyogenic granuloma (lobular capillary hemaniogman |
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What is the cause of 7% of all bening tumors in children
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hemangiomas
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Which type of hemangioma can be seen in Von Hipple Lindau? what other organ is common?
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Cavernous -- common in the liver
-large cavernous vascular channels |
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which is the most common hemangioma, how is it characterized
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the most common is capillary and it is skin and mucous membranes, close packed aggregates of capillary sized vessels
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Describe Juvenile capillary hemangioma
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present at birth, rapid growth then involution (most gone by 7)
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when is pyogenic granulomas most often seen
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they are often seen after trauma or pregnancy --> often ulcerated, polypoid variant of capillary hemangioma
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what are the 4 forms of Kaposi's
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1. chronic/classic/european = older men eastern europe, red purple plaques of LE, relapses and remissions, skin ONLY
2. AIDS related = homosexual males, lesions anywehere in skin/oral & visceral mucosa 3. Lymphadenopathic/African - younge men in equatorial aftrica, restricted to LN, aggressive 4. Transplant associated = cutaneous and visceral involvement, lesions may regress with discontinuation of immmunosuppresion -->MOST AGGRESSIVE Type |
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What are the 3 histological stages of all types of Kaposi's
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Patch -->Plaque--> Nodular
histology - spindle shaped blood vessels, plasma cells admixed. infected with HHV-8 |
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Leukocytoclastic Vasculitis
(not really in the notes but in the learning objectives) |
definition; Acute vasculopathic hypersensitivity disorder characterized by leukocytoclasis and fibrinoid necrosis of small venules in the dermis.
presentation: palpable purpura or erythematous macules, usually on dependent parts, especially the lower legs Pathogenesis: Involves deposition of immune complexes in vessel walls with activation of complement:70 ◦results in: ■chemotaxis of neutrophils ■injury to vessel walls with exudation of: ■serum ■erythrocytes ■fibrin |
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scavenger pathway
(LO not really in notes) |
Robbins talks about a scavenger receptor on macrophages (not the regular LDL rec) that helps the macrophage eat oxidized LDL transforming them into foam cells.
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