Rheum: Systemic Vasculitis

Front 1

name 2 large vessel vasculitis

Back 1

giant cell (temporal) arteritis
takayasu's arteritis

Front 2

name 2 medium vessel vasculitis

Back 2

kawasaki's disease
polyarteritis nodosa

Front 3

name small vessel vasculitis
3 immune complex related
3 pauci immune

Back 3

immune complex
- cryoglobulin vasculitis
- henoch schonlein purpura
- SLE/rheumatoid vasculitis
pauci immune
- wegener's granulomatosis
- churg-strauss syndrome
- microscopic polyangitis

Front 4

granulomatous arteritis of aorta and major branches, esp extracranial branches of carotid

Back 4

giant cell (temporal) arteritis

Front 5

Sx of giant cell arteritis

Back 5

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

Front 6

what is polymyalgia rheumatica(PMR)?

Back 6

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

Front 7

Tx of PMR

Back 7

high dose corticosteroids w/ temporal arteritis
NSAID w/o temporal arteritis or low dose cortico.

Front 8

granulomatous inflammation of aorta and major branches

Back 8

takayasu's arteritis

Front 9

Sx of takayasu's

Back 9

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?

Front 10

medium sized or small arteritis w/o glomerulonephritis or vasculitis in arterioles capillaries or venules

Back 10

polyarteritis nodosa

Front 11

Sx of polyarteritis nodosa (PAN)

Back 11

often associated w/ hep B
skin nodules
mononeuritis multiplex (wrist, foot drop_
frequently involves mesenteric arteries

Front 12

arteritis involving large, medium and small arteries (aorta and veins may be involved).. mainly medium vessels

Back 12

kawasaki's disease

Front 13

Presentation of kawasaki's

Back 13

usually in children
mucocutaneous lymph node syndrome
**coronary arteries often involved
skin desquamation
strawberry tongue

Front 14

vasculitis w/ IgA dominant immune deposit

Back 14

henoch-schonlein purpura

Front 15

presentation of HSP

Back 15

typically involves skin, gut, and glomeruli
associated w/ arthralgia or arthritis
not uncommon in children

Front 16

vasculitis w/ cryoglobulin immune deposits; serum cryoglobulins

Back 16

essential cryoglobulinemic vasculitis

Front 17

presentation of cryoglobulinemic vasculitis

Back 17

skin and glomeruli often involved
association w/ hep C

Front 18

granulomatous inflammation of respiratory tract w/ necrotizing vasculitis

Back 18

wegener's granulomatosis

Front 19

presentation of wegener's

Back 19

typically upper and lower airway w/ glomerulonephritis
pulmonary-renal syndrome

Front 20

necrotizing vasculitis w/ few or no immune deposits

Back 20

microscopic polyangiitis

Front 21

presentation of microscopic polyangiitis

Back 21

larger arteries may be affected
necrotizing glomerulonephritis very common
pulmonary involvement occurs often

Front 22

organ involvement in small vessel vasculitis

Back 22

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

Front 23

which two small vessel vasculitis don't normally present w/ lung or ENT involvement?

Back 23

HSP
cryoglobulinemia

Front 24

what do you call serum Ab reactive w/ alcohol fixed neutrophils?

Back 24

anti neutrophil cytoplasmic antibodies (ANCA)
cytoplasmic ANCA (cANCA) - directed against proteinase
perinuclear ANCA (pANCA) - directed against myeloperoxidase

Front 25

cANCA often associated w/?

Back 25

wegener's granulomatosus

Front 26

pANCA often associated w/?

Back 26

microscopic polyangiitis or churg-strauss syndrome

Front 27

DDx of vasculitis

Back 27

infection - viral/infective endocarditis
embolic
malignancy - hairy cell leukemia
drug induced - ergot, cocaine, amphetamines

Front 28

Dx of vasculitis

Back 28

requires biopsy of involved tissue revealing pathologic findings of vasculitis

Front 29

general Tx strategy?

Back 29

immunosuppression
corticosteroids
cytotoxic drugs - cyclophosphamide (esp for wegener's and life threatening vasculitis), methotrexate, azathioprine
biologics
antibiotics?

Front 30

complications of Tx

Back 30

steroid side effect
opportunistic infections
drug toxicities
cyclophosphamide in wegener's
- hemorrhagic cystitis and bladder carcinoma
- bone marrow suppression and myelodysplastic syndromes
- malignancy

Front 31

pathogenesis of vasculitis?

Back 31

largely unknown
idiopathic?
autoimmune?
blood vessel wall injury
infection?

Front 32

eosinophilic granulomatous inflammation of respiratory tract and necrotizing vascilitis

Back 32

churg-strauss syndrome

Front 33

churg-strauss associated with?

Back 33

associcated with asthma and eosinophilia