Vascular Path Robbins Part 3

Question 1

vasculitis- clinical presentation

Answer 1

vasculitis- clinical presentation -infl of vessels

-nonspecific- fever, malaise, arthralgias, myalgias

Question 2

vasculitis- classified as

Answer 2

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

Question 3

major cause of noninfectious vasculitis

Answer 3

local or systemic response

• immune complex deposition
• ANCAs
• antiendothelial cell ab’s
• autoreactive T cells

Question 4

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

Answer 4

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

Question 5

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

Answer 5

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

Question 6

PR3-ANCA- assoc with?

Answer 6

granulomatosis with polyangiitis

Question 7

MPO-ANCA- assoc with?

Answer 7

• microscopic polyangiitis

- Churg-Strauss syndrome

Question 8

possible mech for ANCA vasculitis

Answer 8

• 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

Question 9

granulomatous arteritis- 3 types

Answer 9

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

Question 10

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

Answer 10

• 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

Question 11

Takayasu arteritis- affects who? presentation?

Answer 11

• 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

Question 12

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

Answer 12

• 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

Question 13

PAN (polyarteritis nodosa)- morphology

Answer 13

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

Question 14

PAN- classic presentation

Answer 14

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

Question 15

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

Answer 15

• 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

Question 16

microscopic polyangiitis- affects? assoc with? morphology?

Answer 16

• 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!”)

Question 17

Churg-Strauss Syndrome- assoc with?

Answer 17

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

Question 18

Behcet disease- triad? assoc with?

Answer 18

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

Question 19

Granulomatosis with polyangiitis- involves? assoc with? therapy?

Answer 19

(Wegener granulomatosis)

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

Question 20

Granulomatosis with polyangiitis- clinical presentation

Answer 20

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

Question 21

Thromboangiitis obliterans- involves? affects who? leads to?

Answer 21

(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

Question 22

infectious vasculitis- most common agent

Answer 22

Pseudomonas

-Aspergillus, Mucor

Question 23

Raynaud Phenomenon

Answer 23

• 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

Question 24

primary raynaud phenomenon- induced by?

Answer 24

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

Question 25

secondary raynaud phenomenon

Answer 25

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

Question 26

myocardial vessel vasospasm- caused by?

Answer 26

- 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

Question 27

myocardial vessel vasospasm- can result in?

Answer 27

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

Question 28

varicose veins- what are they? risks?

Answer 28

- 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!!

Question 29

esophageal varices- caused by?

Answer 29

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

Question 30

other varices

Answer 30

- rectum- hemorrhoids | - periumbilicus- caput medusa

Question 31

hemorrhoids

Answer 31

dilation of venous plexus at anorectal jxn - common!! - pain, bleeding, may ulcerate

Question 32

thrombophlebitis- most where?

Answer 32

(also called phlebothrombosis) - venous thrombosis and infl - 90% in deep v's in legs- can be completely asymptomatic!!

Question 33

risk factor for DVT in LE?

Answer 33

- prolonged activity/immobilization- single most important! - systemic hypercoagulability (may also increase risk) - mechanical factors that slow venous return- CHF, pregnancy, obesity

Question 34

most common serious clinical complication of DVT?

Answer 34

-pulmonary embolism- often 1st manifestation of thrombophlebitis

Question 35

migratory thrombophlebitis - assoc with?

Answer 35

(Trousseau sign) - assoc with cancer- hypercoagulability! - mucin-producing adenocarcinomas - venous thromboses appear at 1 site, disappear, and reappear at another site - adenocarcinomas of lung, ovary, pancreas

Question 36

superior vena cava syndrome- caused by? effects

Answer 36

- neoplasms or aortic aneurysms that compress or invade the SVC - dilation of v's of head, neck, arms with cyanosis - pulm vessels may be compressed- resp distress

Question 37

inferior vena cava syndrome-caused by? effects?

Answer 37

- neoplasms that compress or invade IVC - thrombosis of hepatic, renal, or LE v's that moves cephalad - hepatocellular carcinoma and RCC- grow within v's! - obstruction- LE edema, distention of superficial collateral v's of lower abdomen, proteinuria (with renal v involvement)

Question 38

lymphangitis- what is it? caused by? manifested by?

Answer 38

acute infl elicited by the spread of bacterial infections into lymphatics - group A B-hemolytic streptococci- most common - dilated and filled with exudate of neutrophils/monocytes - red, painful subcutaneous streaks and painful enlargement of draining LNs (lymphadenitis)

Question 39

primary lymphedema- due to?

Answer 39

isolated congenital defect or familial Milroy disease (heredofamilial congenital lymphedema) -lymphatic agenesis or hypoplasia

Question 40

secondary or obstructive lymphedema- due to?

Answer 40

blockage of a previously normal lymphatic - malignant tumors - surgical procedures that remove regional groups of LNs - postirradiation fibrosis - filariasis - postinfl thrombosis and scarring

Question 41

persistent edema from cancer- leads to?

Answer 41

- peau d'orange appearance of overlying skin- seen in breast cancers (lymphatics clogged with tumor cells) - chylous ascites (abdomen), chylothorax, chylopericardium- caused by rupture of dilated lymphatics (secondary to obstruction from a tumor)