Vasculitis

Question 1

Answer 1

palpable purpura

Question 2

Answer 2

leukocytic vasculitis

Question 3

what to do if you suspect a vasculitis

Answer 3

1. determine extent of disease - what organs does it effect
2. BIOPSY - or angiogram, but biopsy is better

Question 4

Four types of small vessel vasculitis

Answer 4

1. Henoch-Schoenlen Purpura
2. Wegener’s granulomatosis
3. microscopic polyarteritis/polyangiitis
4. Churg-strauss syndrome

Question 5

skin clues to vasculitis

Answer 5

• palpable purpura
• ulcers - esp in stellate shape
• nodules
• ischemic digits
• livido reticularis, or reticulated purpura

Question 6

Answer 6

reticulated purpura: a clue to deep vascular involvement

Question 7

Answer 7

leukocytoplastic vasculitits

Question 8

Henoch-Schoenlein Purpura - sx

Answer 8

small vessel vasculitis w/

1. dependent skin rash - palpable purpura
2. abdominal pain/bloody diarrhea due to vasculitis of bowel lining
3. glomerulonephritis - sometimes
4. arthralgias

+ fever and malaise

in a child

usually self-limited

Question 9

biopsy findings for Henoch-Scholein purpura

Answer 9

neutrophilic, leukocytoclastic process w/ IgA

Question 10

Wegner’s granulomatosis/ Granulomatosis polyangiitis

Answer 10

small vessel vasculitis

classic traid: granulomatous inflammation of upper airway, lower airway (lung + trachea), and kidney (focal segmental glomerulonephritis)

systemic sx: epistaxis, SOB, evidence of GN

assoc w/ cANCA in 90% (antibodies to serine proteinase 3)

Question 11

Microscopic polyarteritis/polyangiitis

Answer 11

small vessel vasculitis

Wegner’s-like presentation: involves skin, kidney, lungs (diffuse alveolar hemorrhage syndrome)

pANCA (specificity for MPO)

Question 12

Churg-Strauss syndrome

Answer 12

small vessel vasculitis

similar to Wegener’s in pt w/ hx of asthma or atrophy

sx: pulmonary infiltrates, cardiac and neuro features w/ blood eosinophilia

Question 13

polyarteritis nodosa

Answer 13

medium vessel vasculitis - neutrophilic

almost always secondary to Hep B

sx: ANEURYSMS, gut ischemia, renal artery involvement + hypertension, large ulcers, mononeuritis, systemic sx (weight loss, fever)

Question 14

Kawasaki’s disease

Answer 14

medium vessel vasculitis

febrile illness in children under age 6

sx: inflammation of oral mucous membrane, lymphadenopahty, rash, irritability
complication: coronary arteritis – want to prevent this

Question 15

Takayasu’s aortitis

Answer 15

large vessel vasculutis

inflammation of aorta and its branches

initial phase: fever, malaise, weight loss, ischemic sx

later: progressive vascular occlusion, lose pulses
epi: more common in asian women

Question 16

giant cell arteritis

Answer 16

large vessel vasculitis

most common vasculitis in adults > 55

affects extravranial elastic arteries –> headache, facial pain, visual loss from ischemic optic neuropathy

also can present w/ polymyalgia rheumatica or wasting systemic febrile disease

Question 17

Answer 17

biopsy in polyarteritis nodosa - shows inflammed vessel and aneurysm

elastic lamina destroyed

Question 18

Answer 18

polyarteritis nodosa

Question 19

common causes of secondary vasculitis

Answer 19

• other connective tissue disease
• infections: hep B and C, parvovirus, herpes zoster, SBE
• drug induced: cocaine
• malignancy
• cardiac myxoma
• cryoglobulins

Question 20

eye inflammation, sinusitis, lung lesions, hemoproteinuria, gangrene of fingers

dx? lab tests?

Answer 20

dx: Wegner’s/ Granulomatois Polyangiitis
test: cANCA

Question 21

ANCA tests

Answer 21

pattern: cycoplasmic vs. perinuclear

most common antigens: cANCA = serine proteinase 3; pANCA = myeloperoxidase (MPO)

Question 22

ANCA with discordanent antibodies (ex. cANCA w/ MPO)

Answer 22

happens w/ cocaine!

Question 23

difference b/t vasculitis and vasculopathy

Answer 23

vascultitis: INFLAMMATION of vessel wall
vasculopathy: processes that cause vessel obstruction or narrowing that is non-inflammatory
* examples: cholesterol emboli, amyloid, vasospasn, SBE, more….

Question 24

Answer 24

calciphylaxis - one cause of vasculopathy

Question 25

Bechet’s vasculitis

Answer 25

involves veins and arteries

sx: painful oral and genital ulcers, thrombophlebitis, retinal vasculitis, anterior eye disease, encephalitis, rash

Question 26

Cryoglobulinemic vasculitis

Answer 26

cold precipitating antibodies –> small vessel vasculitis

includes GN

assoc w/ Hep C

Question 27

Buerger’s disease

Answer 27

thromboangiitis obliterans

destructive arterial and venous disease of hand and feet –> digit loss

may be a vasculopathy rather than vasculitis

strong association w/ smoking