Vasculitis

Question 0

clues to vasculitis

Answer 0

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

Question 1

vasculitis definition

Answer 1

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

Question 2

causes of primary vasculitis

Answer 2

idiopathic

Question 3

causes of secondary vasculitis

Answer 3

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

Question 4

what lab tests are specific for vasculitis

Answer 4

ANCA

Question 5

only way to confirm vasculitis diagnosis

Answer 5

biopsy

Question 6

types of small vessel vasculitises

Answer 6

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

Question 7

Henoch-Schoenlen Purpura

Answer 7

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

Question 8

bx of Henoch Schoenelen Purpura

Answer 8

neutrophilic, leukocytoclastic vasculitis

IGA

Question 9

polyarteritis nodosa is almost always caused by

Answer 9

hepatitis B infection

Question 10

polyarteritis nodosa symptoms

Answer 10

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

Question 11

what else can present with polyarteris nodosa

Answer 11

weight loss, fever, lack of localizing signs

Question 12

Kawaski’s disease is…

Answer 12

Polyarteris nodosa in children <6

Question 13

symptosm of kawaski’s dz

Answer 13

inflammation of oral mucous membranes, lympahdenopathy, rash, irritability

Question 14

complication in kawaski’s

Answer 14

coronary arteriris

Question 15

main cause of vasculitis in adults over 55

Answer 15

giant cell arteritis

Question 16

what is the patho phys of GCA

Answer 16

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

Question 17

what does GCA primarily affect

Answer 17

extracranial elastic arteries causing..

facial pain, visual loss from ischemic optic neuropathy, painful proximal muscular aching (PR)

Question 18

catchphrase of giant cell arteritis

Answer 18

“pain putting a hat on”

Question 19

tx of GCA

Answer 19

prednisone (excellent response)

Question 20

Takayasu’s aoritis

Answer 20

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

Question 21

pathophys of Takayasu’s

Answer 21

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

Question 22

initial vs late phase of takayasu’s

Answer 22

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

Question 23

microscopic polyarteritis/polyangiitis

Answer 23

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

Question 24

wegner's classic traid

Answer 24

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

Question 25

what else may the pt present with in wegner's

Answer 25

systemic signs epistaxis shortness of breath evidence of GN

Question 26

ancas in mPAN vs wegener's

Answer 26

mPAN- panca | Wegener's-canca

Question 27

Churg Strauss syndrome

Answer 27

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

Question 28

halmarks of churg strauss

Answer 28

pulmonary infiltrates | cardiac and neurologic features w/ blood eosinophilia

Question 29

primary vs secondary raynaud's

Answer 29

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

Question 30

what is secondary raynaud's due to

Answer 30

exagerated vasomotor response to cold or stress

Question 31

tx raynaud's

Answer 31

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

Question 32

Behcet's

Answer 32

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

Question 33

cryoglobulinemic vasculitis

Answer 33

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

Question 34

buerger disease

Answer 34

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