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Flashcards in 2nd Exam: Vasculitis Deck (81)
1

Vasculitis:

group of systemic disease w variable Cx ft

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TF? Vasculitis affects a single organ.

F.

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Causes of vasculitis:

infection or immune-mediated usually

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Pathology of vasculitis:

endo damage, thrombosis/ clot formation, inflammation, fibrinoid necrosis, thickening, narrowing

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How to dx vasculitis:

biopsy, BEFORE corticosteroids, otherwise inflammation may not be present

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Fibrinoid necrosis:

necrosis of vessel wall, not really fibrin, lumen may narrow and change blood flow

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3 mechs of vasculitis:

immune complex forms deposits, ANCA related, T cell mediated (granulomas)

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Consequences of vasculitis:

aneurysms, hemorrhage, edema, ischemia

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Cx aspects of systemic vasculitis:

inc ESR, CRP, acute phase reactants, fever, chills, malaise, weight loss

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What happens after immune complexes form?

lodge in wall, inflammation

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ANCA sf:

antineutrophil cytoplasmic antibody

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How to differentiate bw the types of vasculitis?

1 disease, 1 vessel usually

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Large vessel vasculitis:

Takayasu / giant cell arteritis

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Medium-sized vessel vaculitis;

Kawasaki disease, polyarteritis nodosa

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Small-vessel vasculitis:

H-S purpura and cryoglobulinemic (Goodpasture syndrome, isolated Cutaneous leukoclastic angiitis, microscopic polyangitis, Wegener granulomatosis and Churg-Strauss syndrome)

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Goodpasture syndrome

rare AI d, Ab's that attack lungs and kidneys, isolated

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Temporal (cranial) arteritis:

oft women 65+, large elastic arteries, branches of carotid aa., aorta, temporal a., pathology represents panarteritis (granulomatous inflammation early, fibrosis and nonspecific changes late, granuloma disappears), not entire length of artery, often nodular, swollen, seen visually, all layers thickened

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How to dx temporal (cranial) arteritis:

biopsy

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Early granulomatous inflammation is diagnostic of:

temporal areteritis

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giant cells indicate:

granulomatous reaction

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neutrophils will be found in this region in temporal arteritis:

Below intima

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Cx ft's of temporal arteritis:

high ESR (often lower than 80), throbbing unilateral headache, scalp pain near the artery, fever, visual disturbances later, ophthalmic a. d. - ischemic optic n., oft preceded by amaurosis fugax, (transient loss of vision in 1 or both eyes), blindnesss wo tx

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Tx for temporal arteritis:

corticosteroids, prevents blindness

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Polyarteritis nodosa:

young to middle aged, over 30yo, males 2:1, affects muscular, not elastic aa., transmural, segmental different aged nodules, deposits in vessel wall (Ig and complement), Hep B ag in deposits 30% of time, Hep B ag may be found in blood, vessels, many aa. are affected, smaller diameter vessels, they may bleed

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TF? Polyarteritis nodosa is a granulomatous disease.

F.

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Transmural is aka:

panarteritis

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cell that lacks polyarteritis nodosa, not granulomatous.

giant cell

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Cells present in a polymorphous infiltrate:

PMN's, lymphos

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Possible effects of polyarteritis nodosa:

thrombosis, infarcts, ischemia

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In early lesions of polyarteritis nodosa:

fibrinoid necrosis

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What does necrosis look like in wall of affected vessel?

fibrin

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What happens after PAN w fibrinoid and organizing thrombus?

inflammatory process attacks thrombosis, ongoing disease, many different temporal aspects, one healing while another in chronic phase

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Cx fts of PAN:

Puzzling, can masquerade, polyneuropathy, kideny disease due to arteritis, not glomerulonephritis (hypertension, renal insufficiency), GI d. w hemorrhage or infarction, pulmonary involvement rare

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If the lungs are involved, rule out this:

PAN

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This rash looks like acute RF:

livedo reticularis

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Livedo reticularis:

related to vasculitits, mottled reticulated vascular pattern, lace-like purplish discoloration of the skin, looks a little like rheumatic fever

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erythema marginatum:

circular, serpiginous (wavy margin), erythematous rash

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Focal dilations of vessel:

aneurysm, round protuberence, inflammation

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To dx aneurysm if many organs are involved:

angiogram

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ANCA-assoc vasculopathies:

Wegner's granulomatous, microscopic polyangitis, Churg-Strauss syndrome, antineutrophil antibodies syndrome

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Characteristic ANCA-assoc vasculopathy:

Wegner's granulomatous

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Churg-Strauss syndrome:

asthma, lung d., skin d.

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antineutrophil antibodies syndrome:

Ab's against granules in PMNs, higher Abs w acute or recurrent disease, c-ANCA = diffuse pattern, granules, (Abs vs proteinase 3), p-ANCA = perinucleuar pattern (Abs vs myeloperoxidase (MPO))

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To determine if antineutrophil antibodies syndrome is getting worse:

Ab titer

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This ANCA is Abs to granules, entire cytoplasm of neutrophils appears stained:

c-ANCA

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This ANCA has a perinuclear pattern:

p-ANCA

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To dx p-ANCA:

immunofluorescence microscopy (IFM)

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ANCAs are generated vs enzymes in the:

azurophilic granules of PMNs

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Abs against enzyme in the azurophilic granules of PMNs result in:

degranulation of PMNs (tissue destruction), adherence of polys to endo (tissue destruction), Abs cause PMNs to damage vessels and tissue (tissue and vessel destruction)

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How do degranulated PMNs cause tissue damage?

contain enzymes, stimulate formation of free radical, damage

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Wegner's granulomatosis, preferred name:

Granulomatosiss w polyangitis

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Granulomatosiss w polyangitis:

usually 40yo men, most have + c-ANCA in serum, necrotizing vasculitis, small aa. and vv., vascualar or extra-vascular granulomas

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Temporal arteritis and polyarteritis nodosa both involve what structures?

vessels, not tissues

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Wegener's granulomatosis involves these structures:

vessels OR tissues, i.e., lungs, nose, hard palate, renal disease

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Staining pattern of Wegener's granulomatosis:

C diffusing (ACNAs)

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Cx ft of Wegener's granulomatosis:

Sinuses, lungs, then kidneys: involves sinuses, nasopharynx (95%) - cough, hemoptysis, dyspnea, lung (90%,), kidney disease (77%) - proteinuria, hematuria, failure

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To test for Wegener's granulomatosis:

immunofluorescence microscopy, c-ANCA pattern (diffuse staining bc there are so many granules bound by Abs)

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Large, necrotic, cavitating granuloatous lesions are characteristic of this:

Wegener's lung in Wegener's granulomatosis:

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Cells found in the outer wall of Wegener's lung vessels:

chronic inflammation: lymphos, plasma cells, Giant cells

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Issue related to Wegener's Lung:

vasculitis, granulomas

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To dx Wegener's from a nasal lesion:

biopsy

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Wegener's renal disease is a disease of:

glomerulus, not vessel/ vasculitits

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Are granulomas in the kidney common or rare?

rare

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Wegener's renal diseases:

crescentic/ RPGN (Rapidly progressive glomerulonephritis), glomerular, no immune deposits (pauci-immune vasculitis)

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2 types of Wegener's renal disease:

crescentic or RPGN

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Pauci-immune vasculitits:

associated w min evidence of hypersensitivity upon immunofluorescent staining for IgG, no immune deposits

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Henoch-Schonlein Purpura:

small vessel acute vascularitis with PMNs, 4-7yo, in spring, oft after URI, esp strep, features in common w IgA nephropathy

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Most common systemic vasculitis in children:

small vessel acute vascularitis with PMNs (H-S Purpura)

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Vasculitis that might be triggered by infection:

H-S Purpura

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Are granulomas seen w small vessel acute vascularitis with PMNs?

no

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Cx ft of H-S Purpura:

palpable dot-like purpura (blood in skin), GI pain, diarrhea, bleeding, protein and blood in urine, pulm d and neuropathy rare, often resolve on own

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Rx for H-S Purpura:

corticosteroids

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Pathologoy of H-S purpura:

inc serum IgA, immune-complex deposits (IgA + unknown Ab), acute vasculitits

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To dx H-S Purpura

fluorescence micro
IgA antibodies in wall of vessel, no other vasculitis looks like this

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Progression of H-S purpura

polys, degranulate, apoptosis, disappear, fragment

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Is H-S purupura bigger or smaller than petechiae:

bigger

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H-S purpura is due to:

leukocytoclastic vacsulitis

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leukocytoclastic vasculitis:

acute necrosis, tiny vessels of skin (leads to bleeding), wall infiltrated by PMNs w nuclear dust (clastic), can see nuclei very well, blue dots smaller than nuclei

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vasculitis in the dermis:

leukocytoclastic vasculitis

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H-S purpura renal d.:

glomerulus, deposited throughout body, especially skin

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H-S purpura renal d. looks like:

IgA nephropathy, deposits of IgA in the mesangium