2nd Exam: Vasculitis Flashcards by Tedman McMahon

Vasculitis:

group of systemic disease w variable Cx ft

How well did you know this?

Not at all

Perfectly

TF? Vasculitis affects a single organ.

How well did you know this?

Not at all

Perfectly

Causes of vasculitis:

infection or immune-mediated usually

How well did you know this?

Not at all

Perfectly

Pathology of vasculitis:

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

How well did you know this?

Not at all

Perfectly

How to dx vasculitis:

biopsy, BEFORE corticosteroids, otherwise inflammation may not be present

How well did you know this?

Not at all

Perfectly

Fibrinoid necrosis:

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

How well did you know this?

Not at all

Perfectly

3 mechs of vasculitis:

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

How well did you know this?

Not at all

Perfectly

Consequences of vasculitis:

aneurysms, hemorrhage, edema, ischemia

How well did you know this?

Not at all

Perfectly

Cx aspects of systemic vasculitis:

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

How well did you know this?

Not at all

Perfectly

What happens after immune complexes form?

lodge in wall, inflammation

How well did you know this?

Not at all

Perfectly

ANCA sf:

antineutrophil cytoplasmic antibody

How well did you know this?

Not at all

Perfectly

How to differentiate bw the types of vasculitis?

1 disease, 1 vessel usually

How well did you know this?

Not at all

Perfectly

Large vessel vasculitis:

Takayasu / giant cell arteritis

How well did you know this?

Not at all

Perfectly

Medium-sized vessel vaculitis;

Kawasaki disease, polyarteritis nodosa

How well did you know this?

Not at all

Perfectly

Small-vessel vasculitis:

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

How well did you know this?

Not at all

Perfectly

Goodpasture syndrome

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

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

How to dx temporal (cranial) arteritis:

biopsy

How well did you know this?

Not at all

Perfectly

Early granulomatous inflammation is diagnostic of:

temporal areteritis

How well did you know this?

Not at all

Perfectly

giant cells indicate:

granulomatous reaction

How well did you know this?

Not at all

Perfectly

neutrophils will be found in this region in temporal arteritis:

Below intima

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

Tx for temporal arteritis:

corticosteroids, prevents blindness

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

TF? Polyarteritis nodosa is a granulomatous disease.

Transmural is aka:

panarteritis

cell that lacks polyarteritis nodosa, not granulomatous.

giant cell

Cells present in a polymorphous infiltrate:

PMN's, lymphos

Possible effects of polyarteritis nodosa:

thrombosis, infarcts, ischemia

In early lesions of polyarteritis nodosa:

fibrinoid necrosis

What does necrosis look like in wall of affected vessel?

fibrin

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

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

If the lungs are involved, rule out this:

PAN

This rash looks like acute RF:

livedo reticularis

Livedo reticularis:

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

erythema marginatum:

circular, serpiginous (wavy margin), erythematous rash

Focal dilations of vessel:

aneurysm, round protuberence, inflammation

To dx aneurysm if many organs are involved:

angiogram

ANCA-assoc vasculopathies:

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

Characteristic ANCA-assoc vasculopathy:

Wegner's granulomatous

Churg-Strauss syndrome:

asthma, lung d., skin d.

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))

To determine if antineutrophil antibodies syndrome is getting worse:

Ab titer

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

c-ANCA

This ANCA has a perinuclear pattern:

p-ANCA

To dx p-ANCA:

immunofluorescence microscopy (IFM)

ANCAs are generated vs enzymes in the:

azurophilic granules of PMNs

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)

How do degranulated PMNs cause tissue damage?

contain enzymes, stimulate formation of free radical, damage

Wegner's granulomatosis, preferred name:

Granulomatosiss w polyangitis

Granulomatosiss w polyangitis:

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

Temporal arteritis and polyarteritis nodosa both involve what structures?

vessels, not tissues

Wegener's granulomatosis involves these structures:

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

Staining pattern of Wegener's granulomatosis:

C diffusing (ACNAs)

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

To test for Wegener's granulomatosis:

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

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

Wegener's lung in Wegener's granulomatosis:

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

chronic inflammation: lymphos, plasma cells, Giant cells

Issue related to Wegener's Lung:

vasculitis, granulomas

To dx Wegener's from a nasal lesion:

biopsy

Wegener's renal disease is a disease of:

glomerulus, not vessel/ vasculitits

Are granulomas in the kidney common or rare?

rare

Wegener's renal diseases:

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

2 types of Wegener's renal disease:

crescentic or RPGN

Pauci-immune vasculitits:

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

Henoch-Schonlein Purpura:

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

Most common systemic vasculitis in children:

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

Vasculitis that might be triggered by infection:

H-S Purpura

Are granulomas seen w small vessel acute vascularitis with PMNs?

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

Rx for H-S Purpura:

corticosteroids

Pathologoy of H-S purpura:

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

To dx H-S Purpura

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

Progression of H-S purpura

polys, degranulate, apoptosis, disappear, fragment

Is H-S purupura bigger or smaller than petechiae:

bigger

H-S purpura is due to:

leukocytoclastic vacsulitis

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

vasculitis in the dermis:

leukocytoclastic vasculitis

H-S purpura renal d.:

glomerulus, deposited throughout body, especially skin

H-S purpura renal d. looks like:

IgA nephropathy, deposits of IgA in the mesangium