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CPR 2 Exam 1 > Vascular Pathology > Flashcards

Vascular Pathology Flashcards

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1
Q

What is an AVM?

A

Arteries and veins without intervening capillaries

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2
Q

“Tangle of worms” with pulsatile arteriovenous shunting with high blood flow is indicative of what?

A

AVM

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3
Q

AVM causes what type of cardiac failure?

A

High-output

Due to shunting of blood into venous circulation

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4
Q

In what population does AVM occur more frequently?

A

Males

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5
Q

When is AVM typically diagnosed?

A

Childhood

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6
Q

What is a surgically created AVM?

A

Arteriovenous fistula for hemodialysis or chemotherapy

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7
Q

What AVM can cause hematuria?

A

AVM of bladder

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8
Q

What are symptoms of an AVM of the brain?

A

Seizure

Intracerebral hemorrhage

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9
Q

What is a focal abnormality of an artery due to underlying defect in the media?

A

Berry (saccular) Aneurysm

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10
Q

Where is a berry (saccular) aneurysm typically found?

A

Anterior cerebral artery

Anterior communicating artery

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11
Q

What are risk factors for berry (saccular) aneurysm?

A

HTN

Smoking

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12
Q

Berry (saccular) aneurysms are associated with which diseases?

A

AD Polycystic kidney disease
Marfan syndrome
Ehlers Danlos syndrome

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13
Q

Subarachnoid hemorrhage is associated with what?

A

Berry (saccular) aneurysm

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14
Q

What are symptoms of a subarachnoid hemorrhage?

A 
Neck pain
Vomiting
Double vision
Seizures
LOC
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15
Q

Berry (saccular) aneurysm rupture often happens with what?

A

Straining with stool

Sexual orgasm

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16
Q

What is a dilation of artery due to infectious process that damages the vessel wall?

A

Mycotic aneurysm

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17
Q

What are the three causes of mycotic aneurysm?

A

1) Septic emboli (infective endocarditis)
2) Extension of an adjacent infectious process
3) Circulating organisms directly infecting the arterial wall

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18
Q

What is a focal, irregular thickening of medium and large muscular arteries?

A

Fibromuscular dysplasia

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19
Q

What arteries does fibromuscular dysplasia typically impact?

A

Renal
Carotid
Splanchnic
Vertebral

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20
Q

Fibromuscular dysplasia is upregulated in women due to what?

A

Estrogen

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21
Q

Fibromuscular dysplasia shows what on angiography?

A

String of beads

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22
Q

What artery does fibromuscular dysplasia most commonly impact?

A

Renal artery

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23
Q

Renal artery stenosis in fibromuscular dysplasia leads to activation of what?

A

RAAS

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24
Q

Activiation of RAAS in renal artery stenosis results in what?

A

Renovascular HTN (bruit sometimes heard)

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25
What maintains blood volume and vascular tone?
Renin-angiotensin-aldosterone system
26
When is renin released?
Low volume Low peripheral resistance Decreased glomerular filtration rate
27
Where is renin released?
By juxtaglomerular cells in the afferent arterioles in the kidneys
28
Renin cleaves circulating angiotensinogen to form what?
Angiotensin I
29
Angiotensin I is cleaved to form angiotensin II by what?
ACE
30
Angiotensin II does what?
Vasoconstrict
31
Volume expansion caused the myocardial cells to release what?
Atrial natriuretic peptide
32
What does atrial natriuretic peptide cause?
Sodium excretion Vasodilation Decreased BP
33
HTN is a risk factor for what?
Atherosclerosis | End organ damage
34
What are the unmodifiable risk factors for primary HTN?
Age (older) | Genetics (blacks, higher risk)
35
What are the modifiable risk factors for primary HTN?
Stress Obesity Physical inactivity Increased salt consumption
36
What are the renal causes of secondary hypertension?
``` Renovascular disease Renal artery stenosis Fibromuscular dysplasia Atherosclerosis Polycystic kidney disease ```
37
What are the endocrine causes of secondary hypertension?
Primary aldosteronism Cushing syndrome Pheochromocytoma
38
What are the cardiovascular causes of secondary hypertension?
Coarctation of the aorta
39
Primary hyperaldosteronism causes HTN with what?
Hypokalemia
40
What symptoms are associated with the HTN due to primary alderosteronism?
Neuromuscular changes (weakness, muscle cramps, paraesthesias, visual disturbances)
41
What are the three causes of hyperaldosteronism?
Bilateral nodular hyperplasia of adrenal gland Adenoma Hybrid glomerulosa cells responsive to ACTH
42
What are the four causes of Cushing syndrome?
Tumor in anterior pituitary Tumor in adrenal Paraneoplastic Cushing Iatrogenic Cushing syndrome (oral steroids)
43
What is a pheochromocytoma?
Tumor of the chromaffin cells of the adrenal medulla
44
What is secreted by a pheochromocytoma?
Epinephrine | Norepinephrine
45
Norepinephrine causes what?
Alpha-1 activation -> peripheral vasoconstriction | Beta-1 activation -> increased cardiac output
46
Epinephrine causes what?
Beta-1 activation -> increased cardiac output
47
What are the clinical symptoms of pheochromocytoma?
``` Paroxysm of elevated blood pressure Tachycardia Palpitations Headache Diaphoresis Tremor ```
48
How do you diagnose pheochromocytoma?
Elevation of urinary or plasma metanephrines
49
What syndrome is pheochromocytomas assocaited with?
MEN 2
50
What do pheochromocytomas look like on gross examination?
Golden brown color
51
What is MEN 1?
Pituitary adenoma Parathyroid hyperplasia Pancreatic tumors
52
What is MEN 2A?
Parathyroid hyperplasia Medullary thyroid carcinoma Pheochromocytoma
53
What is MEN 2B?
Mucosal neuromas Marfanoid body habitus Medullary thyroid carcinoma Pheochromocytoma
54
What is the rule of 10s for pheochromocytomas?
10% are malignant 10% are extraadrenal 10% of sporatic are bilateral 10% are not associated with hypertension
55
What happens with renal artery stenosis?
HTN Decreased GFR Chronic kidney disease Increased creatinine
56
What are the two causes of renal artery stenosis?
Atherosclerosis | Fibromuscular dysplasia
57
What is adult coarctation of the aorta?
Congenital narrowing of the aorta opposite the ligamentum arteriosum
58
What does adult coarctation of the aorta cause?
HTN in upper extremities Hypotension in the lower extremities Limb claudication
59
What is coarctation of the aorta in adults associated with?
Bicuspid aortic valve
60
Coarctation of the aorta is most common in what population?
Males
61
When is a bruit heard?
Significant obstruction of a larger vessel
62
Where is a bruit heard with renal artery stenosis associated with fibromuscular dysplasia?
Epigastric abdominal bruit which radiates to the back or side
63
Where is a bruit heard with coarctation of the aorta?
Carotid bruit
64
Pyelonephritis and polycystic kidney disease can be a secondary cause of what?
HTN
65
How does parenchymal kidney disease cause hypertension?
Activation of the renin-angiotensin system due to renal ischemia
66
Why does concentric left ventricular cardiac hypertrophy occur?
Left ventricle is attempted to pump blood against increased vascular resistance
67
Concentric left ventricular cardiac hypertrophy leads to what?
Cardiomegaly (which then leads to heart failure, which then leads to fluid back up in the lungs, which then leads to dyspnea)
68
What are heart and aorta effects due to HTN?
Cardiac hypertrophy Chronic heart failure Ischemic heart disease Aortic dissection
69
What are kidney effects due to HTN?
Proteinuria
70
What are brain and eye effects due to HTN?
Multi-infarct dementia Cerebrovascular hemorrhage/strone Increased intracranial pressure/papilledema (headache/vision changes) Retinopathy (AV nicking)
71
What do patients with untreated HTN die from?
Ischemic heart disease/CHF | Stroke
72
Hyaline arteriosclerosis due to what?
Increased smooth muscle matrix synthesis and plasma protein leakages across damaged endothelium
73
Which drug class is hyaline arteriosclerosis seen in?
Calineurin inhibitors (T-cell immunosuppressive drug)
74
What is the hyaline material composed of in hyaline arteriosclerosis?
Precipitated plasma proteins | C3
75
Hyaline arteriosclerosis can lead to what downstream?
Ischemia
76
Homogenous pink (hyaline) thickening of the vessel wall leads directly to what?
Luminal narrowing
77
Hyaline arteriosclerosis manifests as what in the kidney?
Hyaline nephrosclerosis
78
Hyaline nephrosclerosis causes what?
Impairment of renal blood supply Ischemic glomerulosclerosis Leads to RAAS activation -> HTN
79
What is a hypertensive crisis?
Systolic greater than 180-200 | Diastolic greater than 120
80
What is a hypertensive emergency?
Hypertensive crisis with end organ damage
81
What end organ damage is seen in hypertensive emergencies?
``` Renal failure Encephalopathy Acute heart failure Retinal hemorrhages and exudates Papilledema ```
82
Papilledema is typically found with what?
Increased CSF pressure
83
What does the kidney show in hypertensive emergencies?
Necrotizing arteriolitis Hematuria Increased creatinine Renal failure
84
What is malignant hypertension?
Markedly increased hypertension
85
Hyaline arteriolosclerosis occurs with what type of hypertension?
Chronic
86
Hyperplastic arteriolosclerosis occurs with what type of hypertension?
Severe
87
In hyperplastic arteriolosclerosis the smooth muscle forms what?
Concentric lamellations with a thickened reduplicated basement membrane
88
"Onion skinning" is found in what type of arteriolosclerosis?
Hyperplastic
89
What accompanies lamellations in malignant hypertension?
Fibrinoid deposits | Vessel wall necrosis (especially in the kidney)
90
Hyaline arteriolosclerosis causes what kidney appearance?
Cortical scarring Shrunken kidney with granular surface Thinned cortex
91
Hyperplastic arteriolosclerosis causes what kidney appearance?
Numerous petechial hemorrhages
92
What does arteriosclerosis mean?
Hardening of the arteries Arterial wall thickening Loss of elasticity
93
What are the three types of arteriosclerosis?
1) Arteriolosclerosis 2) Atherosclerosis 3) Monckeberg medial sclerosis
94
What are the two types of arteriolosclerosis?
Hyaline | Hyperplastic
95
In what vessels does arteriolosclerosis occur?
Small arteries and arterioles
96
What are complications of arteriolosclerosis?
Downstream ischemic injury
97
What happens in atherosclerosis?
Atheromatous plaque formation
98
What are complications with atherosclerosis?
``` Stenosis/occlusion Plaque rupture Aneurysm Thrombus Atheroembolism Microemboli ```
99
What is Monckeberg medial sclerosis?
Calcification of muscular arteries and internal elastic membrane
100
What type of arteriosclerosis is an age related degenerative process?
Monckeberg medial sclerosis
101
What is the number one cause of death in the US?
Myocardial infarction
102
What are the nonmodifiable risk factors for aterosclerosis?
Genetic abnormalities Family history Increasing age Male gender
103
What is the genetic inheritance for atherosclerosis?
Multifactoral inheritance (AD= familial hypercholesteremia)
104
At what age is atherosclerosis most common?
40-60s | Increasing age= increasing risk
105
What is a protective effect for atherosclerosis?
Premenopausal estrogenized women (only younger women)
106
What is the most important factors for developing atherosclerosis?
Genetic predisposition
107
Are risk factors for atherosclerosis synergistic or additive?
Synergistic
108
What are modifiable risk factors for atherosclerosis?
``` Hyperlipidemia Hypertension Cigarette smoking Diabetes Metabolic syndrome Inflammation Hyperhomocysteinemia Lipoprotein a Factors affecting homeostasis Lack of exercise Competitive, stressful lifestyle Obesity ```
109
Which cholesterol delivers cholesterol to peripheral tissues?
LDL
110
Which cholesterol mobilizes cholesterol from the periphery?
HDL
111
How do statins work?
Inhibit HMG-CoA reductase (enzyme key in producing LDL cholesterol) Decreases LDL, increases HDL
112
How can HDL be increased?
Exercise | Moderate alcohol intake
113
What decreased HDL?
Smoking | Obesity
114
What increases LDL?
``` Animal products Trans fat (fried, snack food) ```
115
What decreases LDL?
Soluble fiber | Statins
116
How do cigarettes promote atherosclerosis?
ROS and free radicals cause endothelial dysfunction and platelet activation through oxidization of LDL
117
How does diabetes promote atherosclerosis?
Sugar causes oxidative stress which damages blood vessels and increases inflammation
118
How does hyperhomocysteine increase aterosclerosis?
Oxidative stress leading to damage of the endothelium
119
C-reactive protein correlates with what?
Inflammatory activity | Cardiac risk
120
Pack per day for years doubles risk of what?
Ischemic heart disease
121
Diabetes doubles your chances of what?
Myocardial infarction
122
Hyperhomocysteinemia is associated with what?
Increased coronary atherosclerosis Increased peripheral vascular disease Increased stroke
123
What cytokine is associated with c-reactive protein?
IL-6
124
What makes up metabolic syndrome?
``` Abdominal obesity Increased triglycerides Low HDL Hypertension Insulin resistance ```
125
What is function of endothelium?
Non-reactive barrier that keeps blood and plasma going throughout the body smoothly in laminar flow
126
What causes endothelial activation?
``` Turbulent flow Hypertension Cytokines Complement Bacterial products Lipid products Advanced glycation end-products Hypoxia Acidosis Viruses Cigarette smoke ```
127
What happens in activated endothelium?
Increased expression of procoagulants Adhesion molecules and proinflammatory factors Altered expression of chemokines, cytokines, and growth factors
128
Activated endothelium is the key to driving the pathogenesis of what?
Atherosclerosis
129
What are the steps in vascular injury?
1) Recruitment of smooth muscle cells or smooth muscle precursor cells to intima 2) Smooth muscle cell mitosis 3) Elaboration of extracellular matrix
130
What are the two parts of an atherosclerotic plaque?
Fibrous cap | Necrotic center
131
What makes up the fibrous cap of an atherosclerotic plaque?
``` Smooth muscle cells Macrophages Foam cells Lymphocytes Collagen elastin Proteoglycans Neovascularization ```
132
What makes up the necrotic center of an atherosclerotic plaque?
Cell debris Cholesterol crystals Foam cells Calcium
133
Where is fibrous cap located in the atherosclerotic plaque?
Adjacent to the lumen
134
What is the atherosclerotic plaque surrounded by?
Internal elastic lamina
135
What happens when vessels become completely occluded?
Neovascularization
136
What lipid is atheromatous plaques primary consisted of?
LDL (which accumulates in the intima)
137
When monocytes take up oxidized LDL what cytokine is released to recruit additional monocytes?
IL-1
138
Where does atherosclerosis typically occur?
Branch points | Posterior abdominal aorta
139
What are the steps for atherosclerosis?
1) Chronic endothelial injury 2) Endothelial dysfunction 3) Macrophage activation with smooth muscle recruitment 4) Engulfment of lipid with fatty streak deposition 5) Proliferation of smooth muscle with extracellular matrix deposition
140
Which growth factors are important for smooth muscle cell proliferation and extracellular matrix deposition?
PDGF Fibroblast growth factor TGF-alpha
141
What releases PDGF?
Platelets Macrophages Endothelial cells Smooth muscle cells
142
What are the most extensively involved vessels with atherosclerosis?
Abdominal aorta Coronary arteries Internal carotid arteries Circle of Willis
143
What is the earliest gross indication of atherosclerosis?
Superficial flat yellow fatty streaks
144
What are fatty streaks made of?
Lipid filled macrophages on the intimal surface
145
What does severe atherosclerosis look like grossly?
Ulcerated plaques with calcification and associated blood clots
146
What does a fatty streak turn into?
Fibrofatty plaque
147
What causes a fibrofatty plaque to turn into an advanced/vulnerable plaque?
``` Cell death/degeneration Inflammation Plaque growth Remodeling of plaque and wall extracellular matrix Organization of thrombus Calcification ```
148
What are the three clinical outcomes of atherosclerotic changes?
1) Aneurysm and rupture 2) Occlusion by thrombus 3) Progressive plaque growth
149
What causes aneurysm and rupture with atherosclerosis?
Mural thrombosis Embolization Wall weakening
150
What causes occlusion by thrombus with atherosclerosis?
``` Plaque rupture Plaque erosion Plaque hemorrhage Mural thrombosis Embolization ```
151
What causes critical stenosis with atherosclerosis?
Progressive plaque growth
152
What are clinical effects of atherosclerosis on the brain?
Thrombus causes infarction or stroke | Vascular rupture leas to intracerebral hemorrhage
153
What are clinical effects of atherosclerosis on the kidney?
Renal artery stenosis -> HTN Ischemic injury with uremia Renal failure
154
What is the principle cause of abdominal aortic aneurysms?
Atherosclerosis
155
Obstruction of visceral organs due to atherosclerosis causes what?
Chronic mesenteric ischemia nd infarction
156
Peripheral vascular disease causes what?
Claudication | Gangrene
157
Atherosclerosis can cause what in the heart?
Ischemic heart disease -> stenosis -> myocardial infarction
158
What is an excessive locational abnormal dilation of a blood vessel or ventricular wall?
Aneurysm
159
What is a "true" aneurysm?
Intact but thinned muscular wall at the site of dilation
160
What is a "false" aneurysm?
Defect though the wall of the vessel communicating with an extravascular hematoma that freely communicates with the intravascular space (pulsating hematoma)
161
What arises when blood enters a defect in the arterial wall and tunnels between layers?
Arterial dissection
162
Which aneurysm bulges out on one side?
Saccular
163
Which aneurysm bulges out on all sides of the vessel?
Fusiform
164
Where does a ventricular aneurysm occur?
Site of previous MI
165
What is cystic medial degeneration?
Normal elastin of the media is disrupted and replaced with areas of amorphous ground substance
166
What causes cystic medial degeneration?
Marfan Ehlers Danlos Systemic HTN
167
How does systemic HTN cause cystic medial degeneration?
Narrowing of small arterioles which feed the aorta leading to aortic ischemia, scarring, and inadequate ECM synthesis
168
What do vessels with cystic medial degeneration look like in Marfan syndrome?
Elastin fragmentation with areas without elastin fibers that are filled with proteoglycans
169
Marfan syndrome is caused by a mutation in what gene?
FBN1 (fibrillin gene)
170
What does the FBN1 or fibrillin gene do?
Needed for structural integrity of connective tissue
171
Is there increased or decreased TGF-beta in Marfan syndrome?
Increased
172
In Marfan syndrome fibirllin in unable to bind what?
TGF-beta
173
Increased TGF-beta activity weakens what?
Elastic tissue
174
What are symptoms of Marfan syndrome?
``` Tall stature Long fingers Subluxation/dislocation of lenses Pectus excavatum Mitral valve prolapse Aortic aneurysm Aortic dissection ```
175
Ehlers danlos is due to a mutation in what?
Collagen
176
What are symptoms of ehlers danlos?
``` Hyperelastic, fragile skin Joint hypermobility Lens subluxation Abnormal wound healing Widened scars Bruising Mitral valve prolapse Kyphscoliosis Rupture of colon, cornea, large arteries ```
177
What is syphillis caused by?
Spirochete Treponema pallidum
178
What skin lesion is seen on initial infection with syphillis?
Red chancre lesion in the genital or anal area
179
Tertiary syphillis presents after a latency period of how long?
5 years
180
What organ systems does tertiary syphillis impact?
``` Brain (neurosyphillis) Skin Bones Organs (gumma formation) Heart (obliterative endarteritis) ```
181
What is a gumma formation?
Plasma rich inflammatory lesions
182
What is obliterative endarteritis?
Concentric endothelial and fibroblastic proliferation of small vessels
183
Where does obliterative endarteritis occur with tertiary syphillis?
Vaso vasorum (which feeds thoracic aorta causing ischemia and aneurysm formation)
184
What two cardiovascular conditions occur with tertiary syphillis?
Obliterative endarteritis | Aortitis
185
What appearance does an aortic aneurysm due to tertiary syphillis have?
Tree bark appearance (aorta becomes rough and pitted with concomitant calcification)
186
What is the most important risk factor for abdominal aortic aneurysms?
Atherosclerosis
187
What are risk factors for abdominal aortic aneurysms?
Smoking Male Age
188
What is the typical location for abdominal aortic aneurysms?
Below renal arteries, above aortic bifurcation
189
What do abdominal aortic aneurysms typically contain?
Bland laminated mural thrombus
190
What vasculature does abdominal aortic aneurysms impact?
Renal Superior mesenteric arteries Iliac arteries
191
How do the major of AAA patients present?
Aymptomatically
192
In 1/3 of asymptomatic AAA patients there is what?
Pulsatile abdominal mass
193
In 2/3 of asymptomatic AAA patients it is found during what?
Radiology finding or work up for peripheral vascular disease
194
What are the non-ruptured symptoms of AAA?
Pain in abdomen, back
195
What are the ruptured symptoms of AAA?
Severe acute pain Pulsatile abdominal mass Hypotension
196
What is the size of AAA where surgical bypass is considered?
5 cm
197
5-10% of AAA are what type?
Inflammatory with abundant lymphoplasmacytic inflammation
198
What is a chronic inflammatory disease thought to represent an autoimmune disease in which numerous lymphocytes and IgG4 secreting plasma cells are associated with prominent fibrosis?
IgG4 related disease
199
What organs is IgG4 related disease most common?
``` Pancreas (autoimmune pancreatitis) Biliary tree (sclerosing cholangitis) Major salivary glands Orbit of eye Retroperitoneum ```
200
What happens in IgG4 related disease and AAA?
Weakening of the wall with a fibroinflammatory infiltrate
201
What treats IgG4 disease?
Steroids
202
What is seen on histology of IgG4 disease with AAA?
Spindle fibroblastic-type cell proliferation admixed with clusters of plasma cells and lymphocytes
203
What is primary risk factor for thoracic aortic aneurysm?
HTN
204
What are conditions associated with thoracic aortic aneurysms?
Tertirary syphillis Connective tissue disease (Marfan syndrome) Vasculitis (giant cell aortitis, Takayasu)
205
What are clinical symptoms of thoracic aortic aneurysms?
Breathing difficulties Dysphagia Cough (recurrent laryngeal nerve)
206
When is surgical repair or endovascular placement of graft material considered for thoracic aortic aneurysm?
5.5 cm
207
What is the clinical triad for aortic dissection?
1) Abrupt onset of thoracic pain with a sharp, tearing or ripping character 2) Variation of pulse (absence of proximal extremity or carotid pulse) and a greater than 20 mim of mercury BP difference between arms 3) Mediastinal widening on CXR as blood expands the vessel
208
Where is the pain in aortic dissection?
Radiates to the back and move downward as dissection progresses
209
How does death occur with aortic dissection?
Rupture of dissection through the adventitia into the pericardial, pleural, or peritoneal cavities
210
Is cystic medial degeneration associated with aortic dissection?
Yes
211
What are the two caused os aortic dissection?
1) HTN (men, 40-60) | 2) Connective tissue disorders (Marfan syndrome, Ehlers Danlos)
212
A tear in the intima of the aorta causes development of what?
Hematoma
213
How does a hematoma become a dissecting aneurysm?
Hematoma in the media dissects longitudinally
214
What is a double barreled aorta?
Intimal tear further down the aorta that reconnects the false lumen back to the aorta
215
How does a false aneurysm become a chronic dissection?
False channel is endothelized overtime
216
Do chronic dissections have the potential for future rupture?
Yes
217
What is type A dissection?
Proximal intimal tear in the ascending aorta
218
What is the most common type of aortic dissection?
Type A
219
What is a type B dissection?
Proximal intimal tear in the aortic arch or the descending arota
220
Which type of aortic dissection is more prone to external rupture into the mediastinum or pericardial cavity requiring surgical intervention?
Type A
221
How are uncomplicated type B dissections handled?
Medical management
222
What are the large vessel vasculitis?
Giant cell arteritis | Takayasu arteritis
223
What are the medium vessel vasculitis?
Polyarteritis nodosa | Kawasaki disease
224
What are the small vessel vasculitis?
``` Microscopic polyangiitis Wegener granulomatosis Churg-Strauss syndrome SLE vasculitis Henoch-Schonelin purpura Cryoglobulin vasculitis Goodpasture disease ```
225
What is the most common vasculitis in older adults in the US?
Giant cell arteritis
226
What is a T-cell mediated vasculitis that affects the arteries of the scalp and head?
Giant cell arteritis
227
What is the most common feature of giant cell arteritis?
Headache | also facial pain, fever, ocular diagnoses, fatigue/flu like symptoms
228
Ocular symptomatology occurs in half the patients with what?
Giant cell arteritis
229
Why is it important to catch giant cell arteritis quickly?
Can spread to ophthalmic artery and cause permanent blindness
230
What is used to diagnose giant cell arteritis?
Temporal artery biopsy
231
How is giant cell arteritis treated?
High dose corticosteroids
232
What is seen on the temporal biopsy for someone with giant cell arteritis?
Intimal thickening with granulomatous inflammation and fragmentation of the internal elastic lamina
233
Which vasculitis is a granulomatous autoimmune vasculitis associated with certain HLA subtypes?
Takayasu arteritis
234
What populations are primarily affected by Takayasu arteritis?
East Asia India Latin America
235
What age and gender is most commonly associated with Takayasu arteritis?
Female aged 15-40
236
What are the symptoms of Takayasu arteritis?
``` Ocular disturbances Weakened pulses in upper extremities Fatigue HTN Fever Decreased weight ```
237
What is seen on histology for Takayasu arteritis?
Perivascular mononuclear infiltrate which can evolve to marked destruction with fibrosis and giant cells
238
What is a systemic vasculitis of small to medium vessels associated with hepatitis B?
Polyarteritis nodosa
239
What age group is typically affected by polyarteritis nodosa?
Young adults
240
What is the pathophysiology associated with polyarteritis nodosa?
Immune complex mediated disease with Hep B surface antigen/Hep B surface antibody complexes in vessels
241
What vessels does polyarteritis nodosa impact the most?
``` Renal vessels Heart Liver GI tract Peripheral nerves Skin ```
242
What is seen histologically with polyarteritis nodosa?
Fibrinoid necrosis with embedded inflammatory cells within the wall
243
How does polyarteritis nodosa typically present?
``` Rapidly accelerating HTN (renovascular HTN) Abdominal pain Hematochezia Myalgias Peripheral neuritis of the motor neurons ```
244
Polyarteritis nodosa is fatal with renal involvment unless given what therapy?
Immunosuppression
245
What is an acute multisystem vasculitis of large to medium vessels which presents in early childhood and infancy?
Kawasaki disease
246
What are the symptoms of Kawasaki disease?
``` Conjunctivitis Rash (genital) Adenopathy (cervical) Strawberry tongue Hands (palmar erythema and swelling) Burn (fever) ```
247
Kawasaki disease has a preference for which artery?
Coronary artery
248
What causes Kawasaki disease?
Activated T cells (+ monocytes/macrophages) which are provoked by a viral illness
249
What causes death in Kawasaki disease?
Aneurysmal rupture of coronary arteries
250
Depress ventricular function can result in which vasculitis?
Kawasaki disease
251
What is given to treat Kawasaki disease?
Aspirin and IVIG
252
What is another name for Kawasaki disease?
Mucocutaneous lymph node syndrome
253
What is a small vessel vasculitis with upper respiratory involvement, asthma, peripheral blood esosinophilia, and pulmonary infiltrates?
Churg-Strauss syndrome
254
What are found in a biopsy for Churg-Strauss syndrome?
Eosinophils with granulomas
255
What lab finding is positive for Churg-Strauss syndrome?
MPO-ANCAs
256
What organs systems are involved in Churg-Strauss syndrome?
Skin (palpable purpura) GI tract bleeding Renal disease (focal and segmental glomerulosclerosis)
257
What causes death in patients with Churg-Strauss?
Cardiomyopathy/myocarditis with infarction
258
How is Churg-Strauss treated?
Steroids | Cyclophosphamide
259
What is a necrotizing granulomatous vasculitis which affects small to medium sized vessels of the upper and lower respiratory tract including the sinonasal tract and lung?
Granulomatosis with polyangiitis/Wegner's granulomatosis
260
What respiratory tract conditions are seen in granulomatosis with polyangiits?
Pneumonia with infiltrates Chronic sinusitis Nasopharyngeal ulceration
261
What renal conditions is seen with granulmatosis with polyangiitis?
Necrotizing crescentic glomerulnephritis
262
What population is most commonly affected with granulomatosis with polyangiitis?
Middle aged
263
What is the pathogenesis of granulomatosis with polyangiitis?
T-cell hypersensitivity reaction mediated by inhaled environmental or microbial material
264
What serologic test can identify granulomatos with polyangiits?
PR3-ANCA (C-ANCA)
265
What lung findings are seen with granulomatosis with polyangiitis?
Cavities Bleeds Infiltrates
266
What nasal findings are seen with granulomatosis with polyangiitis?
Stuffiness Nose bleeds Saddle nose
267
What is the treatment for granulomatosis with polyangiitis?
Steroids Cyclophosphamide TNF-antagonists
268
What lesions are seen on histology with granulomatosis with polyangiitis in the lung?
Tan, nodular centrally cavitating lesions
269
What lesions are seen on histology with granulomatosis with polyangiitis in the kidney?
Necrotizing crescentic glomerulonephritis
270
Which vasculitis has uniform stage of disease activity in all vessels?
Microscopic polyangiitis
271
What are the symptoms associated with microscopic polyangiitis?
``` Hematuria Proteinuria Hemoptysis Cutaneous purpura Muscle weakness Bowel pain Bleeding ```
272
Is there deposition of immunoglobin in microscopic polyangiitis?
No
273
What is found on histology for microscopic polyangiitis?
Leukocytoclastic picture with neutrophils infiltrating small blood vessels
274
What vessels does microscopic polyangiitis impact?
Arterioles Capillaries Venules
275
Various stages of disease activity are seen within different vessels in what vasculitis?
Polyarteritis nodosa
276
What is the classic triad for Behcet's disease?
1) Oral aphthous ulcers 2) Genital ulcers 3) Uveitis
277
What HLA is Behcet's disease associated with?
HLA-B51
278
What is seen in the GI system with Behcet's disease?
Ulcerations
279
What inflammatory cell is required for diagnosis of Behcet's disease?
Neutrophils
280
How is Behcet's disease treated?
Steroids | TNF-antagonists
281
What is another name for thromboangiitis obliterans?
Berger disease
282
What arteries does thrombangiitis obliterans typically impact?
Tibial artery | Radial artery
283
What occludes the vessel in thromboangiitis obliterans?
Microabscess (granulomatous)
284
What population is thromboangiitis obliterans found?
Smokers > 35 years old
285
What is the pathophysiology of thromboangiitis obliterans?
Hypersensitivty/toxic reaction to cigarette smoke | Anti-endothelial antibodies
286
What ethnic groups are commonly affected with thromboangiitis obliterans?
Israeli Indian Japanese
287
What is the symptom progression for thromoangiitis obliterans?
Raynaud's -> intermittent claudication -> sever pain at rest -> ulceration -> gangrene
288
What is the pain in claudication due to?
Lack of oxygen in tissues
289
Raynaud is secondary to what diseases?
Systemic lupus Scleroderma Thromboangiitis obliterans
290
What provokes an attack of Raynaud's?
Cold temperature Emotion Stress
291
Is primary Raynaud's symmetric or asymmetic?
Symmetric
292
Does primary or secondary Raynaud's spontaneously remit?
Primary
293
The aorta is involved in which vasculitis?
Giant cell arteritis | Takayasu arteritis
294
Eosinophils are rare in which vasculitis?
Giant cell arteritis
295
Eosinophils are required in which vasculitis?
Churg-Strauss
296
Neutrophils are required in which vasculitis?
Behcet disease
297
Granulomas are required in which vasculitis?
Granulomatosis with polyangiitis
298
Thrombosis is required in which vasculitis?
Berger disease
299
PR3-ANCA is positive in which vasculitis?
Granulomatosis with polyangiitis
300
MPO-ANCA is positive in which vasculitis?
Churg-Strauss | Leukocytoclastic vasculitis
301
Which vasculitis is associated with polymyalgia rheumatica?
Giant cell arteritis
302
Which vasculitis is associated with asthma, atopy, and sinusitis?
Churg-Strauss syndrome
303
Which vasculitisis is associated with Hep B?
Polyangiitis nodosa
304
Which vasculitis is associated with young smokers?
Berger disease
305
Which vasculitis is associated with orogenital ulcers?
Behcet's disease
306
What is thought of as a cardiac Raynaud's phenomenon?
Myocardial vessel vasospasm
307
What are the three main causes of myocardial vessel vasospasm?
1) High levels of vasoactive mediators 2) Elevated thyroid hormones 3) Autoantibodies and T-cells in scleroderma
308
What is excessive vasoconstriction of myocardial arteries or arterioles causing ischemia or infarct?
Myocardial vessel vasospasm
309
What are the sources of high levels of vasoactive meditors?
Exogenous (cocaine, caffeine) | Endogenous (epinephrine/norepinephrine in pheochromocytoma)
310
What is Takotsubo cardiomyopathy?
"broken heart syndrome"
311
What is the pathophysiology of Takotsubo cardiomyopathy?
Emotional distress causes sudden surge of catecholamines which stimulates cardiac muscle cells and triggers constriction of coronary arteries (leads to ischemic cardiomyopathy or death)
312
Hyperthyroidism with elevated thyroid hormone increases the sensitivity to what?
Catecholamines (leading to vasospasm)
313
What population is broken heart syndrome seen in?
Elderly women
314
What are varicose veins?
Abnormally dilated twisted veins which occur from prolonged, increased intraluminal pressure leading to incompetence of the venous valves and dilation
315
Which veins do varicose veins typically occur with?
Superficial veins (with stasis, edema, and thrombosis)
316
What is stasis dermatitis?
Ischemia of overlying skin with hemolysis of extravasated RBCs and ulcerations
317
What does the skin look like with varicose veins?
Yellow and shiny (due to extravasated RBCs)
318
Is embolism common with varicose veins?
No
319
Is pruritis associated with varicose veins?
Yes
320
What does portal hypertension lead to?
``` Esophageal varices Splenomegaly Hemorrhoids Distended superficial epigastric veins Caput medusae ```
321
What causes superior vena cava syndrome?
Compression or invasion of the superior vena cava which causes obstruction of blood flow
322
What is the most common cause of superior vena cava syndrome?
Intrathoracic malignancy
323
What are the two most common malignancies that cause superior vena cava syndrome?
Bronchogenic lung carcinoma | Lymphoma
324
What are other causes of superior vena cava syndrome?
Aneurysm AV fistula Mediastinal fibrosis
325
What are the symptoms of superior vena cava syndrome?
Dilation of veins of head, neck, chest, and arms Cyanosis Facial swelling Neurologic manifestations Respiratory distress (pulmonary vessels compressed) Confusion Headaches Exacerbated as the patient bends forward/lies down
326
What causes inferior vena cava syndrome?
Hepatocellular carcinoma Renal cell carcinoma Thrombosis (DVT) Tumoral vein invasion
327
What are the symptoms of inferior vena cava syndrome?
Lower extremity edema | Distension of superficial lower abdominal veins
328
What is a venous thrombosis with an associated inflammatory response due to clot formation?
Thrombophlebitis
329
What is thrombophlebitis usually caused by?
DVT of the leg
330
What are the symptoms of thrombophlebitis?
Edema Cyanosis Erythema Pain
331
What is the most serious potential consequence with thrombophlebitis?
Pulmonary embolism
332
What is the most important risk factor for thrombophlebitis?
Prolonged inactivity/immobilization
333
What are risk factors for thrombophlebitis?
``` Prolonged inactivity/immobilization Hypercoagulability states (clotting factor mutations, tumor, estrogenic states, factor V leiden) Tumor: thrombotic paraneoplastic syndrome; migratory thrombophlebitis (trousseau sign) ```
334
Mucin produdcing adenocarcinomas are thought to be more what?
Thrombogenic
335
What is a paradoxical embolus?
Embolus crosses from venous to arterial circulation (patent ductus arteriosus, AV malformation, ASD)
336
What is the most common cause of lymphangitis?
Group A beta-hemolytic streptococcus
337
How does lymphangitis due to beta-hemolytic streptococcus present?
Erythematous, warm streaking Cellulitis Abscess formation with fever
338
Inflamed lymphatics present as?
Red, painful subcutaneous streaks
339
Lymphadenitis presents as?
Painful enlargement of draining lymph nodes
340
What is lymphedema?
Swelling caused by fluid buildup due to blockage of lymphatics
341
When does lymphedema occur in the axilla?
After axillary dissection
342
What are the primary causes of lymphedema?
Congenital defect | Familial Milroy disease (lymphatic agenesis/hypoplasia)
343
What are secondary/obstructive causes of lymphedema?
Malignant tumor Surgical procedures (radical mastectomy with axillary node dissection) Post-irradiation fibrosis
344
What is peau d'orange?
Draining lymphatics of skin overlying breast cancer filled with tumor cells
345
What is vascular ectasia?
Local dilation of a blood vessel
346
What is nevus simplex?
Simple birth mark caused by a vascular ectasia in the upper dermis which is extremely common
347
Does nevus simplex regress with time?
Yes
348
What is another name for port-wine stains?
Nevus flammeus
349
What are port wine stains?
Progressive actasisias that persist into adulthood
350
What is Sturge-Weber syndrome?
``` Trigeminal nerve facila port wine stain Mental retardation, seizures, hemiplegia Leptomeningial capillary-venous malformation Skull radio-opacitis Eye abnormalities: glaucoma ```
351
A large facial telangiectasia in a child with mental deficiency would be a prototypical clinical presentation of what?
Sturge-Weber syndrome
352
What causes Sturge-Weber syndrome?
Somatic mutations in fetal ectodermal tissue causes inappropriate maturation of blood vessel formation (not heritable)
353
Where does a nevus simplex typically occur?
Forehead Eyelid Nose Upper lip
354
What is a non-neoplastic permanent dilation of small vessels and capillaries?
Telangectasia
355
When is a spider telangiectasia seen?
Increased circulating estrogen levels (mostly seen on face, neck, and upper chest)
356
Which hormone has dilating effects on small vessels causes telangiectasias?
Estrogen
357
What are two conditions with hyperestrogenism?
Pregnancy | Liver Cirrhosis
358
What causes impaired metabolism and inactivation of estrogen leading to increased estrogen?
Liver cirrhosis
359
What is another name for hereditary hemorrhagic telangiectasia?
Osler-Weber-Rendu disease
360
What is the inheritance pattern for hereditary hemorrhagic telangiectasia?
Autosomal dominant
361
What is mutated in hereditary hemorrhagic telangiectasia?
TGF-beta signaling
362
What happens in hereditary hemorrhagic telangiectasia?
Numerous telangectasias | AV malformations
363
What are the symptoms associated with hereditary hemorrhagic telangiectasia?
Nosebleeds GI Hematuria
364
What is a benign vascular tumor characterized by an increased proliferation of blood vessels filled with blood?
Hemangiomas
365
In which populations are hemangiomas common in?
Infancy | Childhood
366
Where are hemangiomas typically found?
Head | Neck
367
Is malignant transformation of hemangiomas common?
No
368
Von Hippel-Lindau has what type of hemangiomas?
Cavernous hemangiomas (vascular lesions in cerebellum, brain stem, retina, pancreas, liver)
369
Where is pyogenic granulomas most common?
Lips | Mouth Fingers
370
What is another name for pyogenic granulomas?
Lobular capillary hemangioma
371
What are some associations with Von Hippel-Lindau?
Angiomatosis Hemangioblastomas Pheochromocytoma Renal cell carcinoma Pancreatic cysts (pancreatic serous cystadenoma) Endolymphatic sac tumor Bilateral papillary cystadenomas of the epididymis or broad ligament of the uterus
372
What is the most common hemangioma?
Capillary hemangioma
373
What is seen with capillary hemangiomas?
Red spot | Thin-walled capillaries, tightly packed together
374
What is a strawberry/congenital/infantile/juvenile hemangioma?
Capillary hemangioma that is present at birth and regresses
375
What is characteristic of cavernous hemangiomas?
Irregular, dilated vascular channels making a lesions with an indistinct border Involves deep tissue, more likely to bleed
376
Pyogenic granuloma is what type of hemangiomas?
Capillary hemangioma
377
Where are pyogenic granulomas most common?
Oral mucosa | Finger
378
What is granuloma gravidarum?
Pyogenic granuloma seen in the gingiva of pregnant women
379
How does congenital juvenile hemangioma/strawberry hemangioma present grossly?
Red bumpy raised plaque
380
How does congenital juvenile hemangioma/strawberry hemangioma present histologically?
Demarcated lobular collections fo blood vessels
381
What is seen histologically with cavernous hemaniomas?
Enlarged vessel spaces filled with blood
382
What is seen histologically with pyogenic granulomas?
Lobulated capillary proliferation of small vessels
383
What are glomus bodies?
Dermal structures which act as natural arteriovenous shunts
384
What is the function of glomus bodies?
Take blood away from the skin surface when the skin is exposed to a cold temperature preventing heat loss
385
What type of cells are glomus cells?
Modified round smooth muscle cells
386
Glomus tumor is what type of tumor?
Mesenchymal
387
What does the glomus tumor look like grossly?
Bluish with predilection for the subungal location under the fingernail
388
What are the symptoms with glomus tumors?
Pain elicited by changes in temperature or tactile stimulation
389
Are glomus tumors benign?
Yes
390
What is a lymphangioma?
Benign dermal neoplasm measuring up to 2cm comprised of endothelial lined spaces
391
What is the key feature of lymphaniomas?
Dilated spaces without RBCs
392
What is the second main types of lymphangioma?
Cystic hygroma (cavernous lymphangioma)
393
What conditions are cystic hygromas associated with?
Turner syndrome Down's Klinefelter syndrome
394
What is the tratment for cystic hygroma?
Surgical excision
395
Where are lymphangiomas found?
Head Neck Axilla
396
Where are carvernous lymphangiomas found?
Neck | Axilla
397
Are cavernous lymphangiomas large or small?
Large (>15 cm)
398
What is a reactive vascular proliferation that is generated in response to Bartonella bacilli?
Bacillary angiomatosis
399
Bartonella hensale causes what?
Cat scratch disease (necrotizing granulomatous lymphadentitis
400
Bartonella quintana causes what?
Human body louse which served as a vector for transmitting trench fever in WWI
401
Bacillary angiomatosis is seen in what populations?
Immunosuppressed (those with HIV, transplant patients)
402
What is seen clinically with bacillary angiomatosis?
Red, smooth papules and nodules
403
What is seen histologically with bacillary angiomatosis?
Lobular proliferation of capillaries and ectatic vessels lined by endothelial cells typically with background inflammatory cells such as neutrophils, lymphcytes, and histocytes
404
What stain is used for bartonella microorganisms?
Warthin-Starry stain
405
How is bartonella angiomatosis treated?
Antibiotics
406
What is a vascular neoplasm that requires infection with HHV-8, a double stranded herpes virus?
Kaposi sarcoma
407
How is Kaposi sarcoma classified?
Intermediate grade | Borderline tumor
408
What are the symptoms of Kaposi sarcoma?
``` Red-purple nodules/plaques Lower limbs Genitals Face Back Lymph nodes GI ```
409
What are the four types of Kaposi sarcoma?
1) Classic (sporadic) 2) Endemic (African) 3) Iatrogenic (immunosuppresssion related) 4) AIDS associated
410
What population does classic/sporadic Kaposi sarcoma affect?
Older men of Mediterranean, Eastern European, and Middle Eastern decent
411
The African endemic variant of Kaposi sarcoma impacts who?
Lymph nodes and viscera of children (HIGH MORTALITY)
412
Iatrogenic Kaposi sarcoma is assocaited with which drugs?
Calcineurin
413
What is the most common types of Kaposi sarcoma in the US?
AIDS associated
414
What are the three stages of Kaposi sarcoma on the skin?
1) Patch stage: red/purple macules 2) Plaques: red, thickened 3) Tumor: nodule formation
415
What is seen on the histology of the patch stage of Kaposi sarcoma?
Dilated channels containing blood with small hyperchromatic nuclei
416
What is seen on the histology of the plaque stage of Kaposi sarcoma?
Infiltrative proliferation of spindled endothelial cells and irregular vascular channels
417
What is seen on the histology of the tumor stage of Kaposi sarcoma?
Nodule mass of spindled cells
418
What is a malignant tumor of vascular origin?
Angiosarcoma
419
What are the risk factors for liver angiosarcoma?
Arsenic Thorotrast Polyvinyl chorlide (PVC)
420
What is arsenic used for?
Agricultureal pesticides Treatment of wood as a preservative pesticide Well water
421
What is thorotrast used for?
Radioactive compound that was utilized as a radiocontrast agent for imaging
422
PVC is what?
Synthetic plastic polymer
423
When can lymphadema predispose someone to lymphangiosarcoma?
Lymphedema after axillary dissection such as in a radical mastectomy procedure for breast cancer
424
Radiation therapy can induce mutations which cause what years after initial therapy?
Angiosarcoma
425
What does angiosarcoma look like histologically?
Well to poorly defined vascular spaces often containing RBCs lined by endothelial cells with cytologic atypia
426
What stains do angiosarcomas show?
CD31 | CD34
427
When is percutaneous coronary intervention used?
Significant coronary artery disease including patients who have had acute coronary syndrome including ST elevation MI, non-ST elevation MI, and unstable angina
428
What are the two types of coronary intervention for significant stenosis by atherosclerotic plaque?
Balloon angioplasty | Endovascular stenting
429
What is given immediately after coronary metallic stents?
Antithromboti drugs
430
1/3 of patients with traditional metal stents what occurs?
Restenosis
431
What drives the in-stent restenosis?
Endothelial injury of the stent on the vessel which drives smooth muscle proliferation and matrix synthesis (drug eluting stent in utilized to reduce local neointimal hyperplasia and proliferative in-stent restenosis
432
What is the anti-restenotic drug used?
Siroliumus
433
What does a balloon angioplasty do?
Compresses and rupture occluding plaque | Aburpt reclosure can result from thrombosis and spasm
434
When are vascular grafts utlized?
Replace or bypass a vascular segment involved by significant atherosclerotic disease
435
When are large synthetic grafts used?
When replacing large vessels like abdominal aortic aneurysm repair
436
What are two examples of vascular grafts?
Coronary artery bypass grafts | Abdominal aortic aneurysm stent grafting
437
What occurs in coronary artery bypass grafting?
Native arteries and/or veins are harvested and used to bypass the diseased atherosclerotic vessel
438
Saphenous veins are easier to harvest and less technically demanding than internal mammary arteries by their failure is?
Higher
439
What occurs in endovascular abdominal aortic aneurysm repaire?
Endovascular graft device is inserted usually using a femoral artery approach
440
What is a CABG?
Coronary artery graft
441
Which artery is used in a CABG?
Internal mammary artery

CPR 2 Exam 1 (20 decks)

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