Term
| what are some categories that vasculitis can be defined by? |
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Definition
| vasculitis can be categorized by size & type of vessels, demographic predelection, clinical features, histology, and most commonly, vasculitis is caused by these 3 factors: immune complexes, antineutrophil cytoplasmic antibodies (ANCA), and anti-endothelial antibodies |
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Term
| can immune reactants and complement be detected in serum or vessels? |
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Definition
| yes, for example, DNA-anti-DNA complexes are found in vessels in SLE (immune reaction is constantly being mounted against self) |
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Term
| what causes 10% of vasculitis? how is this clinically manifested? |
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Definition
| drug hypersensitivity can cause 10% of vasculitis, which is detectable by skin rashes |
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Term
| what are examples of vasculitis associated with viral infection and resultant immune complexes? |
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Definition
| Hep B can often cause PAN (polyarteritis nodosa) |
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Term
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Definition
| ANCA (antineutrophil cytoplasmic antibodies) serve as useful quantitative/qualitative dx markers, as its levels should reflect degree of inflammatory activity. they function to activate neutrophils, which then cause vascular endothelial injury |
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Term
| can antibodies also be directed against the endothelium? |
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Definition
| yes, antibodies to endothelial cells can potentially induce defects in immune regulation, predisposing pts to SLE or kawasaki disease |
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Term
| what are the 3 most common ways that vasculitis can occur? |
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Definition
| immune complexes, ANCA, and anti-endothelial cell antibodies |
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Term
| what part of neutrophils (as well as monocytes and endothelialcells) are ANCA (antineutrophil cytoplasmic antibodies) against? |
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Definition
| ANCA are antibodies against enzymes in the azurophils (primary granules) in neutrophils and lysozymes in monocytes and endothelial cells |
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Term
| what are the 2 categories of ANCA? how are they differentiated? how is this helpful? |
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Definition
| immunofluorescent patterns and their specific target in the neutrophil. c-ANCA is cytoplasmically localized, and it's specific target is proteinase 3 (PR3) p-ANCA is localized in the perinuclear area, and is specific for myeloperoxidase (MPO). this helps you qualitatively and quantitatively assess the inflammation |
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Term
| where does polyarteritis nodosa (PAN) occur? |
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Definition
| PAN is due to immune complex deposition in small or medium sized arteries (arterioles, capillaries, and venules not involved) in the renal (BUN, creatinine abnormalities) or GI areas (bleeding in stool - melana). *not pulmonary vessels* |
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Term
| are pulmonary vessels affected by PAN? |
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Definition
| no, unlike some other diseases |
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Term
| who does PAN usually affect? how does it present? |
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Definition
| young adults who may have acute, subacute, or chronic lesions along with HTN, weight loss, fatigue and melana (biggest clue - positive hemocult, renal/HTN w/otherwise non-specific symptoms) |
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Term
| what is often the cause of death with PAN? |
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Definition
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Term
| how does PAN present microscopically? |
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Definition
| segmental transmural necrotizing inflammation (often at bifurcations, may appear as nodularity on angiograms, transmural means "through the wall"), fibrinoid necrosis, fibrous thickening of the vessel wall (decreased perfusion, GI/renal symptoms - occusions can cause melana, weight loss, renal problems and HTN). all PAN stages can exist in the same or different vessels. |
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Term
| is PAN associated with glomerulonephritis? |
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Definition
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Term
| is PAN associated with *Hep B antigen? |
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Definition
| 30% are, the antigen-antibody complex can be deposited in vessels |
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Term
| is PAN associated with c-ANCA? |
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Definition
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Term
| what can happen if PAN is left untreated? |
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Definition
| it can be lethal (often due to renal involvement) |
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Term
| what are other symptoms seen with PAN that are common to other inflammatory diseases? |
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Definition
| elevated ESR (erythrocyte sedimentation rate) and leukocytosis |
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Term
| what should be visible microscopically with PAN? |
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Definition
| transmural inflammation, some thrombosis of the lumen, necrosis, fibrosis, inflammatory cell gathering, cell debris, and some RBC seen throughout the necrotic wall |
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Term
| what may be visisble on an angiogram of kidney affected by PAN? |
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Definition
| protuberance, nodularity at bifurcations |
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Term
| what does microscopic polyangitis involve? do its lesions correlate in age? |
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Definition
| arteries, capillaries and venules; all lesions of which tend to correlate in age |
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Term
| what is seen clinically with microscopic polyangitis? |
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Definition
| palpable purpura of the skin, mucous membranes, lungs, brain, heart, GI, kidney and muscle. (distrobution is wider than with PAN) |
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Term
| what is often diagnostic for microscopic polyangitis? |
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Definition
| a skin bx is often diagnostic for microscopic polyangitis |
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Term
| are glomerulonephritis and pulmonary capillaritis seen with microscopic polyangitis? |
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Definition
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Term
| what do pts with microscopic polyangitis present with? |
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Definition
| if the lungs are affected: hemoptysis, joints: arthralgia, other organs: abdominal pain, hematuria, proteinuria, hemorrhage, and muscle pain/weakness |
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Term
| what are some common causes of microscopic polyangitis? |
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Definition
| immune rxn to drugs, microorganisms, tumor antigens (secondary complication from tumor) |
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Term
| what is seen in 70% of microscopic polyangitis? |
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Definition
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Term
| how does microscopic polyangitis compare to PAN? |
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Definition
| they are very similar in appearance, and often require a hx to discern between. granulomatous inflammation is absent in microscopic polyangitis, but fragmentation is seen in both microscopi polyangitis and PAN |
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Term
| what skin lesions are seen with microscopic polyangitis? |
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Definition
| palpable purpura, well circumscribed lesions, and dark brown or purple areas |
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Term
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Definition
| an *allergic granulomatosis/angitis which can mimic PAN/microscopic polyangitis. it is due to infiltration of vessels/perivascular tissue by neutrophils and can have GI involvement as well as palpable purpura |
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Term
| what is churg-strauss associated with? |
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Definition
| allergic rhinitis, bronchial asthma, and eosinophila - p-ANCA is seen in 50% of pts |
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Term
| what are primary causes of morbidity/mortality associated with churg-strauss? |
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Definition
| coronary arteritis and myocarditis due to hyperresponsiveness to allergic stimulus |
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Term
| what is unique about churg-strauss in relative to PAN and microscopic polyangitis? |
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Definition
| it can have bronchial involvement |
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Term
| what is giant cell arteritis? |
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Definition
| giant cell arteritis, otherwise known as temporal arteritis is seen in large to small arteries featuring granulomatous inflammation and fragmentation of internal elastic membranes of the temporal, opthalmic, vertebral arteries and the aorta. it may cause permanent blindness |
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Term
| how may pts present with giant cells arteritis? |
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Definition
| opthalmopathy - blindness which can become permanent, and cerebro-vascular problems - vertebral artery, can mimic stroke |
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Term
| how do pts with giant cell arteritis appear macroscopically? |
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Definition
| they may have a very prominent temporal artery, painful to the touch and may resport headache |
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Term
| how do pts with giant cell arteritis present microscopically? |
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Definition
| there is a preponderance of giant cells (which may also be seen in other vasculitis in lower numbers), *internal elastic membrane fragmentation, and intimal thickening |
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