Rheumatology - Vasculitis - Smith Flashcards
what are the 2 large vessel vasculitis diseases?
Takayasu arteritis and Giant Cell (Temporal) Arteritis
what are the 2 medium vessel vasculitis diseases?
Polyarteritis Nodosa and Kawasaki Disease
what are the 3 small vessel vasculitis diseases?
Microscopic Polyangitis, Granulomatosis w/polyangitis (Wegener’s granulomatosis), IgA Vasculitis (Henoch-Schonlein)
what is the variable vessel vasculitis?
Behcet Disease
who is Takayasu arteritis common in?
<50 y/o asian women
what large vessels does Takayasu Arteritis and Giant Cell (Temporal) Arteritis affect?
aorta and it’s major branches
systemic sx’s of Takayasu Arteritis?
elevations of ESR/CRP during the systemic phase
occlusive sx’s of Takayasu Arteritis?
- Claudication
- Pulselessness in carotid, brachial, radial, femoral, arteries (Must check these)
- Bruits
- Renal vascular HTN
what artery off of the aorta is commonly involved in Takayasu Arteritis? what happens?
Subclavian artery
-stenotic lesion proximal to origin of vertebral artery -> subclavian steal syndrome (10mmHg difference in BP b/w arms)
dx of Takayasu Arteritis?
Normochromic normocytic anemia of chronic disease
Elevated ESR, CRP
MRI or Angiogram of arteries
tx of Takayasu Arteritis?
STEROIDS
- initial dose: 60 mg/day
- taper over several weeks by 10mg/day to lowest dose (10mg/day), then take them off it
Other meds: MTX, Cyclophosphamide
Angioplasty or bypass grafts if irreversible arterial stenosis
who gets Giant Cell (Temporal) Arteritis?
> 50 y/o (avg 79/yo) women from Olmstead County, MN or Scandinavia
what artery is involved in Giant Cell Temporal Arteritis?
Temporal artery
what disease is Giant Cell Temporal Arteritis associated with?
Polymyalgia Rheumatica (PMR)
sx’s Giant Cell Temporal Arteritis?
Pt. >50 y/o with:
- abrupt onset of visual disturbances
- polymyalgia rheumatic sx’s (shoulder/hip stiffness)
- unexplained fever or anemia
- ***Jaw claudication (hurts when eating)
- Renal vascular HTN
what is the complication of Giant Cell Temporal Arteritis?
Complication: VISUAL LOSS
criteria for Giant Cell Temporal Arteritis
CRITERIA (3/5)
- > 50 y/o
- New onset HA
- Tender or decr pulse of temporal artery
- ESR >50 mm/hr
- Bx shows necrotizing arteritis with mononuclear cells or granulomatous process with multinucleated giant cells
what is the GOLD STANDARD for dx of Giant Cell Temporal Arteritis?
Temporal artery bx
-***Pts with only sx’s of PMR do NOT require bx
scheduling of temporal artery bx should NEVER interfere with starting ___ b/c pt will become __
steroid tx b/c pt will become blind
what is the ESR level in Giant Cell Temporal Arteritis?
ESR >70 or >100
imaging for Giant Cell Temporal Arteritis
Angiography, Doppler U/S, MRI/A
tx for Giant Cell Temporal Arteritis NOT complicated by visual loss?
Prednisone 60mg/day, for 2 weeks, reduced to 50mg/day and then to 40mg/day at end of month (dose then gradually reduced each 1-2 weeks, but tx can last 12-16 months)
-Low-dose ASA 81mg PO/daily to reduce risk of visual loss, TIA, CVA
tx for Giant Cell Temporal Arteritis with visual loss?
IV METHYLPREDNISOLONE (1000mg IV daily x 3 days), then follow with PO Prednisone 60mg/daily
who gets Polyarteritis Nodosa?
Male, peak in 6th decade
what medium vessels do Polyarteritis Nodosa and Kawasaki disease affect?
- splenic
- renal
- hepatic
- mesenteric
what viruses are associated with the pathogenesis of Polyarteritis Nodosa?
HBV and HCV
which Vasculitis is NOT associated with ANCA/ANCA negative?
Polyarteritis Nodosa
which vasculitis SPARES the lungs, but affects the kidneys?
Polyarteritis Nodosa
sx’s of Polyarteritis Nodosa
HTN (renin mediated b/c affects kidneys)
Peripheral neuropathies (cog dysfunction, seizures, decreased alertness)
Mononeuropathy multiplex affecting nerves (radial, ulnar) w/ both motor and sensory deficits
skin of legs VERY painful and swollen
GI: abd pain (Early sx in w/meneteric arteritis)
what is an early sx of Polyarteritis Nodosa?
GI: abd pain in pts with mesenteric arteritis (bowel infarct rare, but surgical emergency)