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Flashcards in Vasculitis Deck (35):
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vasculitis definition

inflammatory destruction of vessels causing ischemia, dysfunction or embolism through vessel narrowing or embolism

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clues to vasculitis

skin- palpable purpura, ulcers, nodules, ischemic digits, livido reticularis
GNitis
mononeuritis multiplex
dz of the old in the young (like CAD)
systemic dx w/o infection or malig

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causes of primary vasculitis

idiopathic

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causes of secondary vasculitis

occurs with CT disease, infection (hep C, B, parvo, zoster, SBE), drugs, malignancy

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what lab tests are specific for vasculitis

ANCA

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only way to confirm vasculitis diagnosis

biopsy

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types of small vessel vasculitises

henoch-schoenlen purpura
microscopic polyarteritis/polyangitis
wegener's granulomatosis
churg-strassaiul syndrome

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Henoch-Schoenlen Purpura

skin rash in areas of high hydrostatic pressure (legs, arms)
purpura dont blanch
blood diarrhea (vasculitis of bowel lining)
GNitis
arthralgias

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bx of Henoch Schoenelen Purpura

neutrophilic, leukocytoclastic vasculitis
IGA

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polyarteritis nodosa is almost always caused by

hepatitis B infection

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polyarteritis nodosa symptoms

renal artery involvement (HTN)
gut ischemia
deep cutaneous ulcers**
peripheral neuropathy
mononeuritis

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what else can present with polyarteris nodosa

weight loss, fever, lack of localizing signs

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Kawaski's disease is...

Polyarteris nodosa in children <6

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symptosm of kawaski's dz

inflammation of oral mucous membranes, lympahdenopathy, rash, irritability

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complication in kawaski's

coronary arteriris

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main cause of vasculitis in adults over 55

giant cell arteritis

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what is the patho phys of GCA

mononuclear cell infiltrate destroys elastic lamina of muscular arteries--> become tender, non-elastic, and non-pulsatile

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what does GCA primarily affect

extracranial elastic arteries causing..
facial pain, visual loss from ischemic optic neuropathy, painful proximal muscular aching (PR)

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catchphrase of giant cell arteritis

"pain putting a hat on"

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tx of GCA

prednisone (excellent response)

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Takayasu's aoritis

related to GCA, but more common in younger pts, asians, F>M

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pathophys of Takayasu's

affected vessels are more proximal--hallmark is dz is aorta and its branches

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initial vs late phase of takayasu's

initial- inflam! fever, malaise, weight loss, ischemic symptoms
later- progressive vascular occlusions w/o signs of systemic inflammation

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microscopic polyarteritis/polyangiitis

wegner's like in presentation--typically involves skin, kidneys, lungs w/ diffuse alveolar hemorrhage syndrome
no extravascular granulomas

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wegner's classic traid

vasculitis and extravascular granulomatous inflam in
-upper airways (nose, sinuses, ears)
-lower airways (lungs, trachea)
-kidney (pauci-immune gnitis)

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what else may the pt present with in wegner's

systemic signs
epistaxis
shortness of breath
evidence of GN

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ancas in mPAN vs wegener's

mPAN- panca
Wegener's-canca

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Churg Strauss syndrome

another wegner's like dz in patients with a hx of asthma or atrophy

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halmarks of churg strauss

pulmonary infiltrates
cardiac and neurologic features w/ blood eosinophilia

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primary vs secondary raynaud's

primary- disease of young
secondary- adults with other dz (systemic sclerosis, CREST, SLE, DM, smoking)

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what is secondary raynaud's due to

exagerated vasomotor response to cold or stress

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tx raynaud's

-avoid triggers (cold- gloves, smoking, stress, sympathomimetics, coffee)
-Ca channel blockers (nifedipine)
-sidenafil

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Behcet's

peculiar involving veins as well as arteries--associated with intensely painful oral and genital ulcers, thrombophelbitis, retinal vasculitis, and anterior eye diz, encephalitis and rash

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cryoglobulinemic vasculitis

cold ppt antbodies causing a small vessel vasculitis inducing GN most strongly a/w hep B

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buerger disease

aka thromboangitis obliterans
-obliterative arterial and venous disease of the hand and feet felt by some of the vasculopathy rather than a true vasculitis
-->causes digit loss and closely related to cigarette smoking