Vasculitis Syndromes

Question 1

what are the 3 vasculitis syndromes we are learning about

Answer 1

Polyarteritis nodosa (PAN)
Giant cell arteritis/Temporal Arteritis
Takaysu’s Arteritis

Question 2

what happens in a vasculitis

Answer 2

inflammation and damage to the BVs
the vessel lumen is usually compromised and this is associated with ischemia of the tissues supplied by the involved vessel

Question 3

how is vasculitis syndrome classified

Answer 3

as affecting the small, med, or lg vessels

Question 4

what is the accepted theory?

Answer 4

the deposition of immune complexes in the vessel walls results in compromise of the vessel lumen with ischemic changes in the tissues supplied by the involved vessels

Question 5

what size vessels does PAN affect

Answer 5

small and medium

Question 6

involvement with which arteries is common in PAN

Answer 6

renal arteries
visceral arteries
***characteristic

Question 7

what two diseases can PAN be associated with

Answer 7

hairy cell leukemia

significant HTN

Question 8

where do the lesions usually affect the arteries in PAN

Answer 8

at the bifurcation and branching of the arteries

Question 9

which type of cell infiltrates the vessel wall and perivascular areas, resulting in intimal proliferation and degeneration of the vessel wall (PAN)

Answer 9

polymorphonuclear neutrophils

Question 10

what comes along with necrosis of the vessel (PAN)

Answer 10

compromise of the lumen, thrombosis, or infarction of the tissue

Question 11

as the lesions heal, that is deposited in the vessel which may cause further occlusion of the lumen? PAN

Answer 11

collagen deposition

Question 12

what sort of dilations are characteristic of PAN

Answer 12

aneurysmal dilations

Question 13

what nonspecific signs and symptoms may someone with PAN present with

Answer 13

fever, weight loss, malaise, weakness, h/a, abdominal pain, myalgias

Question 14

what involvement is MC with PAN

Answer 14

RENAL involvement. . manifests as HTN, hemorrhage (in the form of microanurysms), or renal insufficiency

Question 15

organ systems involved with PAN (from most to least common)

Answer 15

renal - htn, failure
MS - arthritis, arthralgia, myalgia
PNS
GI
Skin (raynauds too)
Cardiac
GU
CNS

Question 16

what are the diagnostic serologic tests for PAN

Answer 16

there are none

Question 17

what labs should you get for PAN

Answer 17

normochromic normocytic anemia
cbc - increased leukocyte count with neutophil predom
elevated ESR
other labs that have to do with the organ involvef

Question 18

how do you get the diagnosis for PAN

Answer 18

biopsy of involved organ (provides highest diagnostic yield)

arteriographic demonstration of involved vessels (aneurysms, stenotic segments of involved vessels)

Question 19

what is the treatment for PAN

Answer 19

prednisone and cyclophosphamide

Question 20

what does death usually result from in a person with PAN

Answer 20

bowel infarct, bowel perf, CV causes

Question 21

what size arteries does giant cell arteritis affect?

Answer 21

medium and large

Question 22

WHICH arteries are characteristically involved in giant cell arteritis

Answer 22

one or more branch of the carotid artery (particularly temporal)

also the aorta and its branches

Question 23

what disease is giant cell closely associated with

Answer 23

polymyalgia rheumatica

Question 24

what are the clinical manifestations of polymyalgia rheumatica

Answer 24

stiffness, aching, pain in the muscles of the neck, shoulders, lower back, hips, and thighs
increased ESR

Question 25

what is the tx for PMR

Answer 25

low dose prednisone

Question 26

what is panarteritis (in giant cell)

Answer 26

inflammatory mononuclear cells infiltrate the vessel wall with frequent giant cell formation and there is proliferation of the intima

Question 27

how does giant cell present

Answer 27

fever, anemia, high ESR, headaches in a patient >50 years old

may also have malaise, weight loss, fatigue, sweats, arthraligias
may palpate a tender, thickened, rope-like temporal artery
jaw claudication
ischemis optic neuropathy (can lead to blindness)
1/3 can have lg vessel manifestations as primary presentation— SUBCLAVIAN STENOSIS - ARM CLAUDICAITION

Question 28

how do you diagnose giant cell

Answer 28

elevated ESR (you can follow this)
normochromic, normocytic anemia

clin presentation - fever, anemia, high ESR, >50 yo, +/- headache, PMR sx
diagnosis confirmed by TA biopsy

Question 29

what is the pharmacotherapy for giant cell

Answer 29

prednisone 60mg x 1 mo.. followed by gradual tapering.
most pts req tx for >2 yrs

ASA 81 mg can reduce the occurance of cranial ischemic complications. should be added if no contraindications

Question 30

what is takaysu’s arteritis

Answer 30

medium and large arteries, with a strong predilection for the aortic arch and its branches

Question 31

what population do we see takaysu in

Answer 31

adolescent firls and young women

Question 32

how does the path differ for giant cell versus takayasu (panarteritis)

Answer 32

for giant cell you have proliferation of the intima

takaysu- proliferation and fibrosis of intima and scarring of the media (thus luminal narrowing)

Question 33

what is the presentation of takaysu

Answer 33

malaise, fever, night sweats, arthralgias, anorexia, weight loss

absent pulses of the involved vessels
HTN

Question 34

lab data takayasu

Answer 34

elevated ESR, mild anemia

Question 35

example of takaysu

Answer 35

young woman with decreased peripheral pulses, discrepancies in BP and arterial bruits

Question 36

how is the diagnosis of takaysu confirmed

Answer 36

aortic arteriography or MRA (irregular vessel walls, vessel stenosis, aneurysm formation, vessel occlusion)

Question 37

what is the treatment for takaysu

Answer 37

glucocorticoid approach

consider surgical or arterioplastic approach