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33 Cards in this Set
- Front
- Back
name 2 large vessel vasculitis
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giant cell (temporal) arteritis
takayasu's arteritis |
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name 2 medium vessel vasculitis
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kawasaki's disease
polyarteritis nodosa |
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name small vessel vasculitis
3 immune complex related 3 pauci immune |
immune complex
- cryoglobulin vasculitis - henoch schonlein purpura - SLE/rheumatoid vasculitis pauci immune - wegener's granulomatosis - churg-strauss syndrome - microscopic polyangitis |
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granulomatous arteritis of aorta and major branches, esp extracranial branches of carotid
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giant cell (temporal) arteritis
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Sx of giant cell arteritis
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patient usually >50 (if younger, rule out)
associated with polymyalgia rheumatica involves temporal artery may lead to blindness (retinal ischemia) associated w/ headache, jaw or tongue claudication |
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what is polymyalgia rheumatica(PMR)?
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non specific weakness of shoulder girdle without evidence of muscle inflammation. pain limits muscle use
disease of elderly elevated ESR subset of patients have biopsy proven temporal arteritis |
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Tx of PMR
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high dose corticosteroids w/ temporal arteritis
NSAID w/o temporal arteritis or low dose cortico. |
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granulomatous inflammation of aorta and major branches
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takayasu's arteritis
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Sx of takayasu's
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usually <50 yo
common in japan **frequently present w/ pulseless disease ro vascular insufficiencies of upper extremities may affect any area of aorta. female more common? |
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medium sized or small arteritis w/o glomerulonephritis or vasculitis in arterioles capillaries or venules
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polyarteritis nodosa
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Sx of polyarteritis nodosa (PAN)
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often associated w/ hep B
skin nodules mononeuritis multiplex (wrist, foot drop_ frequently involves mesenteric arteries |
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arteritis involving large, medium and small arteries (aorta and veins may be involved).. mainly medium vessels
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kawasaki's disease
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Presentation of kawasaki's
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usually in children
mucocutaneous lymph node syndrome **coronary arteries often involved skin desquamation strawberry tongue |
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vasculitis w/ IgA dominant immune deposit
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henoch-schonlein purpura
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presentation of HSP
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typically involves skin, gut, and glomeruli
associated w/ arthralgia or arthritis not uncommon in children |
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vasculitis w/ cryoglobulin immune deposits; serum cryoglobulins
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essential cryoglobulinemic vasculitis
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presentation of cryoglobulinemic vasculitis
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skin and glomeruli often involved
association w/ hep C |
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granulomatous inflammation of respiratory tract w/ necrotizing vasculitis
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wegener's granulomatosis
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presentation of wegener's
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typically upper and lower airway w/ glomerulonephritis
pulmonary-renal syndrome |
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necrotizing vasculitis w/ few or no immune deposits
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microscopic polyangiitis
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presentation of microscopic polyangiitis
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larger arteries may be affected
necrotizing glomerulonephritis very common pulmonary involvement occurs often |
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organ involvement in small vessel vasculitis
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skin: leukocytoclastic vasculitis
kidney: glomerulonephritis lung: pulmonary infiltrates or hemorrhage neurologic: inflammation of vaso nervorum leading to nerve infacrtion and polyneuropathy GI: non specific nausea, vomiting, diarrhea musculo skeletal: non specific myalgias, arthralgias, arthritis |
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which two small vessel vasculitis don't normally present w/ lung or ENT involvement?
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HSP
cryoglobulinemia |
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what do you call serum Ab reactive w/ alcohol fixed neutrophils?
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anti neutrophil cytoplasmic antibodies (ANCA)
cytoplasmic ANCA (cANCA) - directed against proteinase perinuclear ANCA (pANCA) - directed against myeloperoxidase |
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cANCA often associated w/?
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wegener's granulomatosus
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pANCA often associated w/?
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microscopic polyangiitis or churg-strauss syndrome
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DDx of vasculitis
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infection - viral/infective endocarditis
embolic malignancy - hairy cell leukemia drug induced - ergot, cocaine, amphetamines |
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Dx of vasculitis
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requires biopsy of involved tissue revealing pathologic findings of vasculitis
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general Tx strategy?
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immunosuppression
corticosteroids cytotoxic drugs - cyclophosphamide (esp for wegener's and life threatening vasculitis), methotrexate, azathioprine biologics antibiotics? |
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complications of Tx
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steroid side effect
opportunistic infections drug toxicities cyclophosphamide in wegener's - hemorrhagic cystitis and bladder carcinoma - bone marrow suppression and myelodysplastic syndromes - malignancy |
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pathogenesis of vasculitis?
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largely unknown
idiopathic? autoimmune? blood vessel wall injury infection? |
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eosinophilic granulomatous inflammation of respiratory tract and necrotizing vascilitis
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churg-strauss syndrome
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churg-strauss associated with?
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associcated with asthma and eosinophilia
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