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Flashcards in Vascular Path Robbins Part 3 Deck (41)
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1

vasculitis- clinical presentation

vasculitis- clinical presentation -infl of vessels
-nonspecific- fever, malaise, arthralgias, myalgias

2

vasculitis- classified as

-noninfectious- mediated by immunologic injury (immune complex depositions, autoab's)- indicates immunosuppressive therapy!!
-infectious- pathogenic organisms invading the vessel wall

3

major cause of noninfectious vasculitis

local or systemic response
-immune complex deposition
-ANCAs
-antiendothelial cell ab's
-autoreactive T cells

4

immune complex vasculitis- what is it? may be seen in?

-deposition of ag-ab complexes in vascular walls- infl rxn occurs
-systemic immunologic diseases (SLE)
-drug hypersensitivity
-secondary to exposure to infectious agent

5

antineutrophil cytoplasmic ab's (ANCAs)- 2 types, act what?

-anti-proteinase-3 (PR3-ANCA; c-ANCA)
-anti-myeloperoxidase (MPO-ANCA; p-ANCA)
-ANCA titers
-act neutrophils- release ROS

6

PR3-ANCA- assoc with?

granulomatosis with polyangiitis

7

MPO-ANCA- assoc with?

-microscopic polyangiitis
-Churg-Strauss syndrome

8

possible mech for ANCA vasculitis

-drugs or cross-reactive microbial ag's induce ANCA formation
-infection, endotoxin exposure, or infl stimulus- elicits cytokines that upreg the surface expression of PR3 and MPO on neutrophils
-ANCAs react with cytokine-act cells
-ANCA-act neutrophils- cause tissue injury

9

granulomatous arteritis- 3 types

-Giant cell (temporal) arteritis
-Takayasu arteritis
-Granulomatosis with polyangiitis

10

Giant Cell (Temporal) Arteritis- affects who? morphology?

-most common vasculitis in older pts
-chronic (T-cell mediated) infl of a's, esp temporal a's
-medial granulomatous infl, w/ multinucleated giant cells
-fragmentation of elastic lamina
-patchy and focal
-healed sites- scarring of medial, intimal thickening
-ophthalmic a- vision loss

11

Takayasu arteritis- affects who? presentation?

-similar morphology to giant cell arteritis
-aortic arch and major branch vessels ("pulseless disease")
-pulm, coronary, renal a's may be involved
-younger age group! (<50)
-weak pulse, low BP in UE's

12


PAN (polyarteritis nodosa)- involves? age group? assoc with? therapy?

-systemic vasculitis, immune complex mediated
-renal vessels, heart, liver, GI tract
-pulm vessels spared!!
-classically young adult!
-chronic hepatitis B- HBsAg-HBsAb complexes- 1/3 of pts
-immunosuppressive therapy

13

PAN (polyarteritis nodosa)- morphology

-transmural necrotizing infl- small/medium a's
-fibrinoid necrosis of vessel wall
-typically not circumferential!!
-susceptible to thrombus formation/occlusion, aneurysm, rupture

14

PAN- classic presentation

-young adult
-rapidly accelerating HTN due to renal involvement
-abdominal pain and bloody stools- vascular GI lesions
-diffuse myalgias

15

Kawasaki disease- age? involves? presenting picture? treatment?

-infants/small children (<4 yrs)
-coronary a's- can form aneurysms- thrombosis/rupture- acute MI!!
-erythema of conjunctiva, oral mucosa, palms, and soles; cervical LN enlargement ("mucocutaneous LN syndrome")
-usually self-limited
-IVIg and aspirin- lowers risk of coronary event

16

microscopic polyangiitis- affects? assoc with? morphology?

-necrotizing vasculitis of arterioles, capillaries, venules
-many organ systems- renal glomeruli and lung capillaries most common!
-MPO-ANCA (most cases)
-segmental necrotizing infl with fibrinoid necrosis
-apoptotic neutrophils ("leukocytoclastic vasculitis!")

17

Churg-Strauss Syndrome- assoc with?

-Eosinophils!!
-small vessel necrotizing vasculitis- assoc with asthma, allergic rhinitis, hypereosinophilia
-eosinophils and granulomas!
-<1/2 show ANCA positivity
-many organ systems

18

Behcet disease- triad? assoc with?

-vasculitis of small/medium vessels
-aphthous ulcers of oral cavity, genital ulcers, uveitis!!
-neutrophilic
-HLA-B51

19

Granulomatosis with polyangiitis- involves? assoc with? therapy?

(Wegener granulomatosis)
-necrotizing vasculitis; granulomas
-upper/lower resp tracts, focal necrotizing often crescentic glomerulonephritis!!
-PR3-ANCA (95%)
-immunosuppressive therapy

20

Granulomatosis with polyangiitis- clinical presentation

-males, age 40
-persistent pneumonitis and sinusitis, renal disease, nasopharyngeal ulceration
-if untreated- 80% mortality in 1 yr

21

Thromboangiitis obliterans- involves? affects who? leads to?

(Buerger Disease)
-thrombosing vasculitis!- tibial, radial a's!!
-almost always smokers, young adults
-leads to vascular insuff of extremities
-may involve adj v's and n's- pain!
-chronic ulcerations- gangrene

22

infectious vasculitis- most common agent

Pseudomonas
-Aspergillus, Mucor

23

Raynaud Phenomenon

-vasospasm of small a's and arterioles, esp in fingers and toes (sometimes nose, earlobes, lips)
-digits- "red, white, and blue" from proximal to distal

24

primary raynaud phenomenon- induced by?

-induced by cold or emotion
-symmetric involvement of digits
-young women; 3-5% of population
-benign course

25

secondary raynaud phenomenon

-component of another arterial disease, such as SLE, scleroderma, thromboangiitis obliterans
-asymmetric involvement of digits
-worsens with time!!

26

myocardial vessel vasospasm- caused by?

-vasoconstriction of myocardial a's or arterioles ("cardiac Raynaud"- may cause ischemia or infarct
-circulating vasoactive agents- epinephrine or cocaine
-MI can occur after 20-30 min

27

myocardial vessel vasospasm- can result in?

-sudden cardiac death
-Takotsubo cardiomyopathy ("Broken heart syndrome")- ischemic dilated cardiomyopathy

28

varicose veins- what are they? risks?

-abnormal dilations of v's with valvular incompetence, secondary to sustained intraluminal P
-incompetenence of venous valves- results in stasis, congestion, thrombus, edema, ischemia of overlying skin (stasis dermatitis), ulcerations
-obesity, pregnancy
-embolism from thrombi of superficial LE v's- rare!!

29

esophageal varices- caused by?

-portal HTN (cirrhosis) opens portosystemic shunts which direct blood to v's at the gastroesophageal jxn
-may fatally rupture!!

30

other varices

-rectum- hemorrhoids
-periumbilicus- caput medusa