Vascular Path Robbins Part 3 Flashcards
vasculitis- clinical presentation
vasculitis- clinical presentation -infl of vessels
-nonspecific- fever, malaise, arthralgias, myalgias
vasculitis- classified as
- noninfectious- mediated by immunologic injury (immune complex depositions, autoab’s)- indicates immunosuppressive therapy!!
- infectious- pathogenic organisms invading the vessel wall
major cause of noninfectious vasculitis
local or systemic response
- immune complex deposition
- ANCAs
- antiendothelial cell ab’s
- autoreactive T cells
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
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
PR3-ANCA- assoc with?
granulomatosis with polyangiitis
MPO-ANCA- assoc with?
- microscopic polyangiitis
- Churg-Strauss syndrome
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
granulomatous arteritis- 3 types
- Giant cell (temporal) arteritis
- Takayasu arteritis
- Granulomatosis with polyangiitis
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
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
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
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
PAN- classic presentation
- young adult
- rapidly accelerating HTN due to renal involvement
- abdominal pain and bloody stools- vascular GI lesions
- diffuse myalgias
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
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!”)
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
Behcet disease- triad? assoc with?
- vasculitis of small/medium vessels
- aphthous ulcers of oral cavity, genital ulcers, uveitis!!
- neutrophilic
- HLA-B51
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
Granulomatosis with polyangiitis- clinical presentation
- males, age 40
- persistent pneumonitis and sinusitis, renal disease, nasopharyngeal ulceration
- if untreated- 80% mortality in 1 yr
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
infectious vasculitis- most common agent
Pseudomonas
-Aspergillus, Mucor
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
primary raynaud phenomenon- induced by?
- induced by cold or emotion
- symmetric involvement of digits
- young women; 3-5% of population
- benign course
secondary raynaud phenomenon
- component of another arterial disease, such as SLE, scleroderma, thromboangiitis obliterans
- asymmetric involvement of digits
- worsens with time!!
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
myocardial vessel vasospasm- can result in?
- sudden cardiac death
- Takotsubo cardiomyopathy (“Broken heart syndrome”)- ischemic dilated cardiomyopathy
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!!
esophageal varices- caused by?
- portal HTN (cirrhosis) opens portosystemic shunts which direct blood to v’s at the gastroesophageal jxn
- may fatally rupture!!
other varices
- rectum- hemorrhoids
- periumbilicus- caput medusa
hemorrhoids
dilation of venous plexus at anorectal jxn
- common!!
- pain, bleeding, may ulcerate
thrombophlebitis- most where?
(also called phlebothrombosis)
- venous thrombosis and infl
- 90% in deep v’s in legs- can be completely asymptomatic!!
risk factor for DVT in LE?
- prolonged activity/immobilization- single most important!
- systemic hypercoagulability (may also increase risk)
- mechanical factors that slow venous return- CHF, pregnancy, obesity
most common serious clinical complication of DVT?
-pulmonary embolism- often 1st manifestation of thrombophlebitis
migratory thrombophlebitis - assoc with?
(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
superior vena cava syndrome- caused by? effects
- 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
inferior vena cava syndrome-caused by? effects?
- 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)
lymphangitis- what is it? caused by? manifested by?
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)
primary lymphedema- due to?
isolated congenital defect or familial Milroy disease (heredofamilial congenital lymphedema)
-lymphatic agenesis or hypoplasia
secondary or obstructive lymphedema- due to?
blockage of a previously normal lymphatic
- malignant tumors
- surgical procedures that remove regional groups of LNs
- postirradiation fibrosis
- filariasis
- postinfl thrombosis and scarring
persistent edema from cancer- leads to?
- 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)
