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Block 9 Week 1 > Fung > Vascular Path > Flashcards

Fung > Vascular Path Flashcards

1
Q

what is ECM made of?

A

elastin
collagen
glycosaminoglycans

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2
Q

what are the 3 components of the vasculature?

A

endothelial cells
smooth muscle cells
ECM

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3
Q

what are the 5 fxns of the endothelial cells?

A 
maintain non-thrombogenic blood-tissue interface
modulate vascular resistance
metabolize hormones
regulate inflammation
regulate cell growth
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4
Q

which component of the vasculature proliferates when stimulated?

A

smooth muscle cells

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5
Q

what do smooth muscle cells synthesize?

A

collagen
elastin
proteoglycans

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6
Q

what do smooth muscle cells elaborate?

A

growth factors

cytokines

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7
Q

what are the 3 layers of the vasculature?

A

intima
media
adventitia

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8
Q

what are the 2 specialized layers in muscular arteries?

A

internal elastic lamina

external elastic lamina

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9
Q

T/F: arteries have valves

A

false

veins do

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10
Q

where do berry aneurysms occur?

A

cerebral vessels

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11
Q

T/F: the majority of berry aneurysms occur sporadically

A

TRUE

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12
Q

what are 4 possible genetic causes of berry aneurysms?

A

AD polycystic kidney disease
Ehlers-Danlos syndrome
NF1
Marfan

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13
Q

what are 2 risk factors for developing berry aneurysms?

A

cigarette smoking & HTN

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14
Q

are berry aneurysms present at birth?

A

no
they develop over time
(they’re still inexplicably called congenital though)

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15
Q

what are atriovenous fistulas?

A

small direct connections btwn arteries & veins that bypass capillaries

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16
Q

can inflammatory necrosis of adjacent vessels cause AV fistulas?

A

yes

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17
Q

can developmental defects cause AV fistulas?

A

yes

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18
Q

can penetrating injuries that pierce arteries & veins cause AV fistulas?

A

yes

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19
Q

can rupture of an arterial aneurysm into an adjacent vein cause an AV fistula?

A

yes

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20
Q

can AV fistulas be iatrogenic?

A

yes

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21
Q

what is fibromuscular dysplasia?

A

focal irregular thickening of the walls of medium & large muscular arteries

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22
Q

what does fibromuscular dysplasia result in?

A

luminal stenosis

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23
Q

at what age does fibromuscular dysplasia occur?

A

any

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24
Q

in what group is fibromuscular dysplasia most common?

A

young women

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25
what is an aneurysm?
localized abnormal dilation of a blood vessel or the heart
26
T/F: aneurysms are always acquired
false | they can also be congenital
27
what are the 2 types of aneurysms?
true | false
28
what are the 2 forms of true aneurysms?
saccular | fusiform
29
what is a true aneurysm?
involves an intact attenuated arterial wall or thinned ventricular wall
30
what are 4 examples of true aneurysms?
atherosclerotic syphilitic congenital ventricular following transmural infarction
31
what is another name for a false aneurysm?
pseudo-aneurysm
32
what is a false aneurysm?
a defect in the vascular wall leading to an extravascular hematoma that freely communicates w/ the intravascular space
33
what is an example of a false aneurysm?
ventricular rupture w/ pericardial adhesion
34
what is a saccular true aneurysm?
spherical outpouchings involving only a portion of the vessel
35
how big are saccular true aneurysms?
5-20 cm diameter
36
what is a fusiform true aneurysm?
diffuse circumferential dilation of a long vascular segment
37
how big are fusiform true aneurysms?
up to 20 cm diameter
38
where can you find fusiform true aneurysms?
extensive portions of the aortic arch abdominal aorta iliac arteries
39
what can cause an aneurysm to form (generally)?
any process that causes weakening of the vessel wall
40
what are the 2 main general causes of aneurysms?
sporadic OR connective tissue disease
41
what is Marfan syndrome?
a defect of fibrillin
42
what is Ehlers-Danlos?
a defect in the synthesis or structure of fibrillar collagen
43
what is Vitamin C deficiency?
altered collagen cross-linking
44
what is Loeys-Dietz syndrome?
a defect in elastin, collagen I, & collagen III
45
how can inflammation cause an aneurysm?
alters the balance of synthesis & destruction of collagen | increased matrix metalloproteases (MMP) degrade the ECM
46
what causes a thickening of the intima and cystic medial degeneration?
loss of smooth muscle cells OR proliferation of non-collagenous/non-elastic ECM
47
what is the consequence of a thickened intima?
decreased diffusion of oxygen and nutrients to the media
48
what are the 2 most important predisposing factors for aneurysms?
HTN & atherosclerosis
49
what does atherosclerosis cause particularly?
AAA | abdominal aortic aneurysm
50
what does HTN cause particularly?
ascending aortic aneurysm
51
can infection cause an aneurysm?
yes d/t embolization, direct extension, or organism circulation
52
can aneurysms be congenital?
yes (developmental berry aneurysms)
53
what is the most common location of atherosclerotic aneurysms?
abdominal aorta
54
how do AAAs form?
cystic medial degeneration results in thinning & weakness
55
what is a major influence for developing AAA?
increased MMP (matrix metalloproteases)
56
T/F: AAAs are always saccular
false | they can be saccular or fusiform
57
what is an inflammatory type AAA?
dense periaortic fibrosis containing abundant lymphocytes, plasma cells, & macrophages
58
what is a mycotic type AAA?
d/t circulating microorganisms that destroys the media
59
what are the 4 clinical consequences of a AAA?
rupture w/ potential fatal hemorrhage obstruction of branch vessel embolism from atheroma or mural thrombus impingement on adjacent structures
60
what is a thoracic aneurysm most commonly assoc w?
HTN
61
what are the sx of a thoracic aneurysm?
bone pain cough (pressure on recurrent laryngeal nerve) aortic valve dilation w/ insufficiency
62
what can a TAA encroach on?
mediastinal structures lungs & airways esophagus
63
what is a dissection?
blood splays apart the laminar planes of the media to form a blood filled channel w/i the vessel wall
64
T/F: dissection is always assoc w/ vessel dilation
FALSE (may or may not be assoc w/ it)
65
who gets dissections?
men 40-60 yo w/ HTN | younger pts w/ systemic & localized abnormalities of the aorta
66
why don't you usually see dissection w/ atherosclerosis?
bc of medial fibrosis
67
what is the major risk factor for dissection?
HTN
68
what is the pathophysiology of dissection?
medial hypertrophy of the vasa vasorum w/ degenerative changes of the media i.e. injury d/t diminished flow
69
what 3 tissue disorders can lead to dissection?
Marfan Ehlers-danlos Vitamin C deficiency
70
what is the most frequent histologically detectable lesion w/ dissection?
cystic medial degeneration
71
what is a Type A dissection?
a proximal lesion involving either the ascending aorta only or in conjunction w/ the descending aorta
72
what DeBakey types are assoc w/ Type A dissection?
I & II
73
what is a Type B dissection?
distal lesion beginning distal to the subclavian artery & NOT involving the ascending aorta
74
what DeBakey types are assoc w/ Type B dissection?
type III
75
what is DeBakey type I?
involves ascending AND descending aorta | type A
76
what is DeBakey type II?
involves ascending aorta only | type A
77
what is DeBakey type III?
involves the descending aorta only (distal to subclavian) | type B
78
what is vasculitis?
general term for vessel wall inflammation
79
what do the clinical features of vasculitis depend on?
which vascular bed is affected
80
T/F: vasculitis affects only small vessels
false | affects any vessels of any organ or any size
81
what are the 2 most common pathogenic mechanisms of vasculitis?
immune-mediated inflammation | direct invasion of vascular walls by infectious pathogens
82
T/F: infectious pathogens can initiate both infectious AND non-infectious vasculitis
true | idk why
83
what vessels does large vessel vasculitis affect?
aorta | large branches to extremities/head/neck
84
what are 2 types of large vessel vasculitis?
``` Giant cell (temporal) arteritis Takayasu arteritis ```
85
what vessels does medium vessel vasculitis affect?
main visceral arteries & their branches
86
what are 2 types of medium vessel vasculitis?
Polyarteritis nodosa | Kawasaki disease
87
what vessels does small vessel vasculitis affect?
arterioles venules capillaries small arteries
88
what vessels does variable vessel vasculitis affect?
no predominant type
89
what are 2 types of variable vessel vasculitis?
Behcet's disease | Cogan's syndrome
90
what is the mechanism for large vessel vasculitis?
granulomatous disease
91
what is the mechanism for medium vessel vasculitis?
immune-complex mediated OR anti-endothelial cell ab
92
what are the mechanisms for small vessel vasculitis?
immune complex mediated OR paucity of immune complexes (often w/ ANCA)
93
what are the 4 types of immune-complex mediated small vessel vasculitis?
SLE IgA Cryoglobin OTHER
94
what are the 3 types of "paucity of immune complexes" small vessel vasculitis?
vasculitis w/o asthma or granulomas granulomas, no asthma eosinophils, asthma, & granulomas
95
what are the 8 types of small vessel vasculitis?
``` SLE vasculitis IgA vasculitis cryoglobinemic vasculitis Wegener granulomatosis Churg-Strauss syndrome Microscopic polyantitis anti-GBM disease hypocomplementemic urticarial vasculitis ```
96
what are 3 other types of vasculitis?
single organ vasculitis assoc w/ systemic disease vasculitis assoc w/ probable etiology
97
does non-infectious vasculitis predispose you to vasculitis?
yes | Kawasaki disease
98
what 3 things happen in non-infectious vasculitis?
immune complex deposition anti-neutrophil cytoplasmic ab anti-endothelial cell ab
99
what are 3 examples of immune-complex associated vasculitis?
SLE polyarteritis nodosa drug hypersensitivity vasculitis
100
what 3 things are detected in immune complex assoc vasculitis?
ab complement ag-ab complexes
101
what does ANCA stand for?
antineutrophil cytoplasmic antibodies
102
what is ANCA?
circulating abs react w/ neutrophil cytoplasmic ag
103
what are the abs directed against in ANCA?
``` they're heterogeneous directed against: neutrophil primary granules monocyte lysosomes endothelial cells ```
104
what are the 2 types of antineutrophil cytoplasmic antibodies?
anti-myeloperoxidase (MPO-ANCA) & anti-proteinase-3 (PR3-ANCA)
105
what is MPO?
a lysosomal granule constituent
106
what conditions is MPO-ANCA seen in?
therapeutic agents (propylthiouracil) microscopic polyangitis Churg-Strauss syndrome
107
what is p-ANCA?
perinuclear
108
what is c-ANCA?
cytoplasmic
109
what is PR3?
a neutrophil azurophilic granule constituent
110
what does PR3 share homology with?
microbial peptides
111
when do you see PR3-ANCA?
in Wegener granulomatosis
112
what are the 2 mechanisms for vasculitis with ANCA?
drugs or cross-reactive microbe (ag) induce ANCA formation OR neutrophils release MPO/PR3 & cause ANCA formation in a susceptible host
113
what happens when the host releases cytokines?
causes expression of MPO/PR3 on neutrophils or other cell types
114
what happens when ANCA reacts?
directly induces endothelial injury OR activates other neutrophils
115
what do ANCA-activated neutrophils do?
degranulate, release ROS > further injure endothelial cells
116
what is the process for vasculitis with ANCA (4 steps)?
1. induced ANCA formation via drugs (or ag) OR neutrophil release of MPO/PR3 2. cytokine release > MPO/PR3 expression on neutrophils 3. ANCA react > endothelial injury or activation of neutrophils 4. ANCA-activated neutrophils degranulate > ROS > injure endothelial cells
117
what is the most common vasculitis among elderly pts over 50 yo?
giant cell arteritis
118
what is giant cell arteritis?
chronic granulomatous inflammation of large to small-sized arteries
119
what does giant cell arteritis affect?
arteries of the head: temporal artery, vertebral artery, ophthalmic artery, aorta
120
is giant cell arteritis a medical emergency?
YES | ophthalmic artery involvement can cause blindness
121
what is the mechanism of giant cell arteritis?
T cell lymphocytic immune response to an unknown ag
122
what 2 things may also contribute to giant cell arteritis?
TNF & anti-endothelial cell humoral immune responses
123
what is the histology of giant cell arteritis?
discontinuous involvement of the vessel intimal thickening medial granulomatous inflammation with giant cells elastic lamina fragmentation
124
what is the clinical consequence of having discontinuous involvement of the vessel?
multiple biopsies
125
what is takayasu arteritis?
granulomatous arteritis of medium or large arteries
126
what characterizes takayasu arteritis?
ocular disturbances | marked weakening of pulses in upper extremities (pulseless disease)
127
what happens to the aorta in takayasu arteritis?
fibrous thickening of the aortic arch & great vessels
128
what does takayasu arteritis share features with?
giant cell arteritis | BUT W/ TAKAYASU THE PTS ARE LESS THAN 50 YO!!!!!
129
what is polyarteritis nodosa?
systemic vasculitis of small or medium-sized muscular arteries
130
what arteries does polyarteritis nodosa affect?
``` renal vessels visceral vessels (heart, liver, GI tract) ```
131
what arteries does polyarteritis nodosa NOT affect?
pulmonary circulation | arterioles, capillaries, venules
132
what disease is polyarteritis nodosa assoc w/?
chronic hep B
133
what complexes in vessels w/ hep B & polyarteritis nodosa?
HBsAg-HBsAb | immune complex mediated
134
what is the cause of polyarteritis nodosa?
unknown in most cases
135
what is polyartertitis nodosa?
segmental transmural necrotizing inflammation (neutrophils, eosinophils, & lymphocytes)
136
how does polyarteritis nodosa affect the vessel?
circumferentially
137
what region does polyarteritis nodosa prefer?
vessel branch points
138
what are some complications of polyarteritis nodosa?
weakened vessels | aneurysms
139
what replaces the acute inflammatory infiltrate in polyarteritis nodosa?
fibrous thickening of the vessel wall
140
what are the lesions like in polyarteritis nodosa?
coexist in different stages > recurrent & ongoing insults
141
what is kawasaki disease?
acute febrile self-limited illness of infancy/childhood
142
what vessels does kawasaki affect?
large to medium to small vessels
143
what kind of syndrome is kawasaki?
mucocutaneous lymph node syndrome
144
what are the sx of kawasaki?
``` conjunctival & oral erythema & erosion edema of hands & feet erythema of palms & soles desquamative rash cervical lymph node enlargement ```
145
why is kawasaki clinically significant?
coronary artery involvement
146
what can cause MI in kawasaki?
aneurysms that rupture & thrombose
147
why is there vasculitis in kawasaki?
from delayed hypersensitivity rxn of T cells to uncharacterized ag (possibly infectious agents)
148
what is the auto-ab process in kawasaki?
cytokines produced B cell activation auto-ab produced against endothelial & smooth muscle cells
149
what part of the vessel do kawasaki inflammatory lesions affect?
entire thickness of the vessel wall
150
does kawasaki have more or less fibrinoid necrosis than polyarteritis nodosa?
less
151
what happens w/ healed lesions in kawasaki?
may have obstructive intimal thickening
152
what is microscopic polyangitis?
segmental fibrinoid necrotizing vasculitis
153
what vessels does microscopic polyangitis affect?
capillaries, arterioles, and venules
154
what disease does microscopic polyangitis resemble?
polyarteritis nodosa | but microscopic polyangitis affects SMALLER VESSELS
155
T/F: all lesions in microscopic polyangitis are at the same stage at the same time
TRUE
156
which ANCA is microscopic polyangitis assoc w/?
MPO-ANCA
157
what is another name for microscopic polyangitis?
leukocytoclastic vasculitis
158
what does microscopic polyangitis affect?
``` skin > palpable cutaneous purpura mucous membranes lungs > hemoptysis brain heart GI tract > bowel pain & bleeding kidney > hematuria, proteinuria muscle > pain, weakness ```
159
what can microscopic polyangitis occur w/?
henoch-schonlein purpura essential mixed cryoglobinemia vasculitis assoc w/ CT disorders
160
what is Churg-Strauss syndrome?
small vessel necrotizing vasculitis
161
what is churg-strauss assoc w/?
``` asthma allergic rhinitis lung infiltrates peripheral hypereosinophilia extravascular necrotizing granulomas ```
162
what is churg-strauss also called?
allergic granulomatosis
163
what does churg-strauss histologically resemble?
PAN or microscopic polyangitis | BUT churg-strauss has granulomas & eosinophils
164
what ANCA is present sometimes w/ Churg-Strauss?
MPO-ANCA
165
what are the clinical manifestations of churg-strauss?
palpable purpura GI tract bleeding focal & segmental glomerulosclerosis
166
what 3 things characterize the necrotizing vasculitis assoc w/ Wegener granulomatosis?
acute necrotizing granulomas in upper &/or lower respiratory tract necrotizing granulomatous vasculitis affecting small-med sized vessels focal necrotizing crescentic glomerulonephritis
167
what does Wegener granulomatosis resemble?
polyarteritis nodosa | BUT Wegener has respiratory involvement
168
what is Wegener granulomatosis?
a T-cell mediated hypersensitivity rxn to an inhaled infectious agent
169
what ab it present in 95% of wegener pts?
anti-PR3 c-ANCA
170
what is another name from thromboangiitis obliterans?
buerger disease
171
what is thromboangiitis obliterans?
segmental thrombosing acute & chronic inflammation of medium & small arteries
172
what does thromboangiitis obliterans lead to?
vascular insufficiency principally in the tibial & radial arteries (sometimes veins & nerves)
173
who gets thromboangiitis obliterans?
almost exclusively seen in heavy smokers before 35 yo
174
why do cigarettes cause vascular damage?
directly toxic to endothelial cells OR an immune response to cigarettes
175
what are the clinical features of thromboangiitis obliterans?
superficial nodular phlebitis Raynaud-type cold sensitivity instep claudication severe pain at rest in extremities
176
what is infectious vasculitis?
direct invasion of infectious agents (bacteria or fungus)
177
how can infectious vasculitis spread hematogenously?
seeding during sepsis or embolization
178
what can infectious vasculitis lead to?
mycotic aneurysms
179
what is Raynaud phenomenon a result of?
exaggerated vasoconstriction of digital arteries & arterioles
180
what are the sx of Raynaud phenomenon?
paroxysmal pallor | cyanosis of digits of hands & feet
181
what is primary raynaud phenomenon?
exaggeration of central & local vasomotor responses to cold or emotional stress
182
what is secondary raynaud phenomenon?
``` vascular insufficiency of the extremities secondary to arterial disease caused by... SLE Scleroderma Buerger disease Atherosclerosis ```
183
what are varicose veins?
abnormally dilated tortuous veins
184
how are varicose veins produced?
prolonged increased intraluminal pressure & loss of vessel support
185
where can you get varicose veins?
superficial veins of upper & lower leg esophageal varices hemorrhoids
186
how can varicose veins cause esophageal varices?
d/t portal vein HTN
187
what can esophageal varices do?
rupture > fatal hemorrhage
188
what are thrombophlebitis & phlebothrombosis?
interchangeable names for venous thrombosis & inflammation
189
where do you get thrombophlebitis?
``` deep leg veins periprostatic venous plexus pelvic venous plexus large skull veins dural sinuses ```
190
where does MOST thrombophlebitis occur?
deep leg veins | 90%
191
why do pts get thrombophlebitis in their deep leg veins?
prolonged immobilization predisposes you
192
what can deep leg vein thrombophlebitis lead to?
PE
193
what predisposes you to thrombophlebitis?
systemic hypercoagulability
194
when can you get migratory thrombophlebitis?
paraneoplastic syndromes
195
what causes SVC syndrome?
neoplasms that compress or invade the SVC (bronchogenic ca or mediastinal lymphoma)
196
what are the complications of SVC syndrome?
dilation of the veins of the head | neck & arm cyanosis
197
what causes IVC syndrome?
neoplasms that invade or compress the IVC (HCC, RCC) OR thrombus from hepatic, renal, or LE veins
198
what is lymphangitis?
acute inflammation d/t bacteria
199
which bacteria cause lymphangitis?
group A beta-hemolytic streptococci
200
what causes primary lymphedema?
congenital defects OR familial agenesis or hypoplasia
201
what causes secondary lymphedema?
``` tumors lymph node dissection post-irradiation fibrosis filariasis post-inflammatory thrombosis & scarring ```
202
T/F: primary tumors of large vessels are common
FALSE | they're rare!
203
what is the neoplasm you get in the vessels?
soft tissue sarcoma
204
from what can vessel neoplasms be derived?
endothelium OR cells that support blood vessels
205
what do benign vessel tumors produce?
well-formed vascular channels lined by normal endothelium
206
what do malignant vessel tumors look like?
``` NO well-formed vascular channels cytologic atypia (duh) ```
207
what are hemangiomas/pyogenic granulomas?
common tumors w/ increased #s of BVs filled w/ blood
208
what is a capillary hemangioma?
closely packed thin walled capillaries
209
what is a cavernous hemangioma?
large dilated vascular channels
210
what is a pyogenic granuloma a form of?
capillary hemangioma
211
what is lymphangioma?
benign lymphatic analogues of BV hemangioma
212
what is simple capillary lymphangioma?
small lymphatic channels
213
what is cavernous lymphangioma?
massively dilated lymphatic channels w/ lymphocytes in the CT
214
what is another name for cavernous lymphangioma?
cystic hygroma
215
what is the cause of Kaposi sarcoma?
HHV-8
216
who gets chronic Kaposi sarcoma?
older Eastern European/Mediterranean men
217
how does Kaposi sarcoma present?
skin lesions
218
who gets lymphadenopathic KS?
people in areas of Africa
219
how does lymphadenopathic KS present?
lymphadenopathy (duh)
220
what is transplant associated KS?
occurs in organ transplant & immunosuppressed setting
221
how does transplant associated KS present?
nodal, mucosal, visceral involvement | v aggressive
222
who gets AIDS-assoc KS?
pts w/ AIDS
223
what is the most prevalent malignancy in AIDS pts?
AIDS assoc KS
224
how does AIDS assoc KS present?
lymph node & visceral involvement
225
what is angiosarcoma?
malignant endothelial neoplasms that range from well-differentiated to anaplastic
226
what settings give you hepatic angiosarcoma?
arsenic, thorotrast, polyvinyl chloride, lymphedema, post-radiation
227
what is a glomus tumor?
benign tumor arising from modified smooth muscle cells of the glomus body
228
what is the glomus body involved w/?
thermoregulation
229
what is vascular ectasia?
common lesions but NOT true neoplasms | local dilation of pre-existing vessels
230
what are the types of vascular ectasia?
nevus flammeus spider telangiectasia hereditary hemorrhagic telangiectasia
231
what are some examples of nevus flammeus?
birthmarks port-wine stain Sturge-Weber syndrome
232
what is another name for hereditary hemorrhagic telangiectasia?
Osler-Weber-Rendu disease
233
what is bacillary angiomatosis?
vascular proliferation arising from an opportunistic infection in immunocompromised pts
234
what bacteria causes bacillary angiomatosis?
Bartonella
235
what is hemangioendothelioma?
intermediate neoplasm eg epithelioid hemangioendothelioma
236
what is hemangiopericytoma?
rare tumors derived from pericytes
237
i am so sorry this is a fucking million cards
at least it's over! :)

Block 9 Week 1 (4 decks)

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