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21. CPR Exam 1 > 5. Blood Vessels > Flashcards

5. Blood Vessels Flashcards

1
Q

What are the 3 concentric layers of blood vessels?

A

Intima
media
adventitia

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2
Q

The intima is a single layer of endothelial cells. The media contains well organized layers of smooth muscle in arteries, which are organized haphazardly in veins. The adventitia contains?

A

loose connective tissue containing nerve fibers and vaso vasorum (vessels of the vessels)

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3
Q

There are elastic arteries (aorta), muscular arteries (coronary), and arterioles (tissues/organs) which are the principal point of?

A

physiologic resisitance to blood flow, since halving the diameter increases resistance 16 fold
BP control

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4
Q

Capillaries are small with no media and have pericytes which contract. Veins contain how much total blood volume?

A

2/3 total blood volume

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5
Q

What do arteries have that differentiates them from veins, among other characteristics?

A

internal elastic lamina (these are what break and dilate, causing aneurysms.

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6
Q

What are the 3 vascular anomalies?

A

aneurysms
arteriovenous malformations (AVM)
Fibromuscular dysplasia

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7
Q

What is a localized abnormal dilation of a blood vessel of the heart, which is not present at birth but develop over time due to underlying defect in the media of the vessel?

A

Aneurysms

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8
Q

Arteriovenous malformations shunt blood from arteries to veins without going through capillaries. What type of channels are present with prominent ulsatile ateriovenous shunting with high blood flow?

A

tangle, worm-like

**leads to high output cardiac failure

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9
Q

What is a focal irregular thickening in medium and large muscular arteries (renal carotid, splanchnic, and vertebral) which can be a developmental defect or from trauma?

A

fibromuscular dysplasia

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10
Q

What type of aneurysm is seen in the circle of willis and in AD polycystic kidney dz, with 90% of saccular aneurysms being found near branch points of anterior circulation in the circle of willis?

A

berry (saccular) aneurysms

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11
Q

Berry aneuryms are the most common cause of subarachnoid hemorrhage (SAH). 1/3 occur due to increased ICP (sex/stool). What is the most common location in the circle of willis?

A

anterior communicating artery, then MCA

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12
Q

What may originate from the following?

  1. embolization of septic embolus d/t infective endocarditis
  2. extension of a suppurative process
  3. organisms infecting the arterial wall
A

Mycotic aneurysms (d/t infected artery)

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13
Q

AVMs can result from reupture of aneurysm into vein, injuries that pierce A/V, or from inflammatory necrosis. Why would a procedure be done to make an AVM?

A

Ateriovenous fistulas provide vascular access for chronic hemodialysis

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14
Q

Fibromuscular dysplasia’s focal irregular thickening is common in young women and increased incidence if have first degree relative. What can occur and be seen if it occurs in renal arteries?

A

Renovascular hypertension

string of beads can be seen on angiography d/t attentuation of adjacent media

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15
Q

vascular injury associated with endothelial cell dysfunction or loss, stimulates smooth muscle cell recruitment and proliferation and assocaited matrix synthesis, ULTIMATELY resulting in?

A

intimal thickening

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16
Q

What are motile, undergo cell division, and acquire new biosynthetic capabilities?

A

neointimal smooth muscle cells

*can return to normal but results in intimal thickening

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17
Q

What is the initial event following vascular injury?

A

endothelial cell activation

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18
Q

Sustained BP over 139/89 is associated with increased risk of athersclerosis. What are the three types of HTN?

A

secondary
essential
malignant

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19
Q

5% of the population has secondary HTN, d/t underlying renal or adrenal dz (cushings/pheochromacytoma). HTN secondary to renal artery stenosis is caused by?

A

increased production of renin from the ischemic kidney

*bruit can be heard

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20
Q

Essential HTN is idopathic and 95% have this. It can lead to cardiac hypertrophy (left ventricular) and failure, multi infarct and renal failure. Who is more likely to have idopathic HTN? 2

A

vulnerability increases with age

african americans

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21
Q

Malignant HTN is when BP is 200/120 consistently, causes death in 1-2 years. What are 3 symptoms associated with it?

A

Severe HTN
Renal Failure
Retinal hemorrhage/exudate +/- papilledema

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22
Q

What is a function of cardiac output and peripheral vascular resistancem both of which are influenced by multiple genetic and environmental factors?

A

Blood pressure

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23
Q

Blood volume and vascular tone are modified and maintained by renin-angiotensin-aldosterone system (RAAS). In states of low volume or low peripheral resistance or decreased GFR, what occurs?

A

renin is released by juxtaglomerular cells in afferent arterioles in the kidneys

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24
Q

Renin cleaves circulating angiotensinogen to form angiotensin I. AGT1 is cleaved to form AGTII via?

A

ACE angiotensin converting enzyme

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25
Q

AGTII is potent but short lived vasoconstrictor. AGTII stimulates adrenal cortex to release aldosterone, causing?

A

renal absorption of sodium and water

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26
Q

AGTII is potent but short lived vasoconstrictor. AGTII stimulates adrenal cortex to release aldosterone, causing?

A

renal absorption of sodium and water

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27
Q

Since the kidney is absorbing sodium and water, resisitance and volume increase, raising blood pressure. The volume expansion induces myocardial release of atrial natriuretic peptide, which does what?

A

leads to Na+ excretion and diuresis, as well as vasodilation — lowering blood pressure

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28
Q

Hypertension affects 30% of adults in the US, prevalence of HTN increases with age and being african american. HTN is a major risk factor for? 3

A

atherosclerosis
CHF
renal failure

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29
Q

HTN is controlled by a single gene disorder or is secondary to renal, adrenal or other endocrine disorders. Sustained HTN requires participation of?

A

The kidney

primary hyperaldosteronism is MC secondary cause HTN

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30
Q

HTN causes degenerative changes in the walls of large and medium arteries that can lead to aortic dissection and or cerebrovascular hemorrhage. What are the two forms of SMALL blood vessel dz?

A

Hyaline arteriolosclerosis

Hyperplastic arteriolosclerosis

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31
Q

Hyaline arteriolosclerosis causes increase in smooth muscle matrix synthesis and shows what, causing luminal narrowing?

A

homogeneous pink (hyaline) thickening of the vessel wall

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32
Q

In nephrosclerosis due to HTN, arteriolar narrowing of hyaline arteriosclerosis causes diffuse impairment of renal blood supply and?

A

glomerular scarring

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33
Q

Hyperplastic arteriolosclerosis occurs in severe HTN, smooth muscle cells form _____ which leads to luminal narrowing.

A

concentric lamellations (onion skinning)

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34
Q

In malignant hypertension laminations are accompanied by fibrinoid deposits and vessel wall necorsis in the kidney called?

A

necrotizing arteriolitis

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35
Q

a Granular arteriolosclerotic kidney can be seen in benign HTN along with hyalinization or onion skinning of the arteriole. What can be seen in malignant HTN?

A

Numerous variegated hemorrhages and necrosis of glomeruli

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36
Q

Arteriosclerosis is when the arterial wall thickens and losses elasticity. While arteriolosclerosis may cause down stream?

A

ischemic injury (hyalinization/hyperplastic changes)

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37
Q

What occurs in people older than 50, with calcification of muscular arteries, internal elastic membrane involved, but no narrowing of the lumen and no clinical significance?

A

Monckeberg medial sclerosis

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38
Q

What is known as gruel and hardening; the most frequent and clinically important pattern?

A

Atherosclerosis

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39
Q

Atherosclerosis underlies the pathogenesis of coronary, cerebral, and peripheral vascular dz. It causes more morbidity and mortality than any other disorder. What is responsible for 1/4 of all deaths?

A

myocardial infarction

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40
Q

Atheromas or atherosclerotic plaque is a raised lesion with a soft grumous core of lipid covered by a fibrous cap. Besides obstructing flow, what else can this do?

A

plaques can rupture and cause obstructive vascular thrombosis

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41
Q

Plaques also increase the diffusion distance from the lumen to the media, causing ischemic damage resulting in?

A

weakening of the vessel wall and possible aneurysm formation

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42
Q

Athromas have a fibrous cap in the intima which contains smooth muscle cells, Mø, foam cells, collagen, elastin and neovascularization. What can be found in the necrotic center? 3

A

cell debris

  • cholesterol crystals (cleffs)
  • *foam cells - foamy mø
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43
Q

(men) 40-60 y/o have 5x increased incidence, with multiple risk factors having synergistic effects. What is said about atherosclerosis and premenopausal women?

A

They are protected, postmeno estrogen is not proven to help

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44
Q

Hypercholesterolemia is a risk factor for atherosclerosis. Metabolic syndrome (central obesity) conditions such as insulin resistance, HTN, dyslipidemia (high LDL low HDL), hypercoagulobility and pro inflammatory state may contribute to?

A

endothelial dysfunction and or thrombosis

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45
Q

What is the MCC of left ventricular hypertrophy?

What is the most important risk factor for atherosclerosis?

A

Chronic HTN

Family history

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46
Q

What is the simplest and most sensitive marker of inflammation?

A

C reactive protein CRP

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47
Q

CRPs expression is increased by a number of inflammatory mediators, (IL6) and it augments innate immune response by binding to bacteria and activating?

A

classical complement cascade

more CRP= inc. risk of MI, stroke, sudden cardiac death

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48
Q

The response to injury model views atheroslcerosis as a chronic inflammatory and healing response of the arterial wall to endothelial injury. The two most important causes of endothelial dysfunction are?

A

hemodynamic turbulence and hypercholesterolemia (circulating lipids)

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49
Q

hemodynamic turbulence occurs at openings of exiting vessels, branch points and what?

A

posterior abdominal aorta

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50
Q

Lipids in atheromatous plaques are predominantly cholesterol and cholesterol esters which accumulate in the intima and are taken up by?

A

macrophages and partially oxidized

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51
Q

The modified lipid.LDL accumulates within mø and smooth muscle cells forming what, and a lesion known as a fatty streak?

A

foam cells – this stimulates an inflammatory response to accumulation of this toxic form of LDL

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52
Q

List the steps in the response to injury hypothesis (5)

A
  1. Chronic endothelial injury (hyperlipidemia)
  2. Endothelial dysfunction (inc. perm = monocyte adhesion and emigration)
  3. Macorphage activation/SM recruitment
  4. Mø and SM cells engulf lipids = Foam cell
  5. SM prolife, collagen/matrix deposition
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53
Q

Chronic inflammation contributes to the intiation and progression of atherosclerotic lesions. Accumulation of cholesterol crystals within mø recognized by the inflammosome leads to what secretion?

A

IL1, more mø and T cells are recruited and activated at the site

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54
Q

Smooth muscle proliferation and matrix deposition leads to atherosclerosis as well. Several growth factors are implicated in the proliferation of SM, including PDGF, fibroblast growth factor and?

A

TGF-a

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55
Q

SM cells synthesize ECM and collagen. Due to intimal expansion from foam cells and extracellular lipids, inflam cells, SM cells, and increased ECM, what is formed?

A

antheromatous plaque!

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56
Q

From most frequent/severe sites of athersclerosis to least: abdominal aorta, coronary arteries, popliteal, ICA, and circle of willis. What are 4 complications of atherosclerotic plaques?

A

rupture and ulceration (thrombosis)
hemorrhage into plaque
Embolism
aneurysm formation

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57
Q

What are fatty streaks composed of?

A

lipid filled foamy macrophages

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58
Q

Consequences of atherosclerosis include stenosis of the arterial lumen with continuously growing plaques. The lumen of the affected vessel shrinks, leading to ischemia a point known as ?

A

critical stenosis where approx 70% of the vessel is occluded leading to chronic ischemia

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59
Q

An acute thrombus may form over the plaque, occluding the artery. This may occur secondary to rupture of the plaque or?

A

erosion or ulceration of the plaque surface (or hemorrhaging into the atheroma expanding its volume)

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60
Q

There are vulnerable and stable plaques determined by the amount of collagen in the fibrous cap, which is continuously degraded and resynthesized. What can degrade the cap?

A

inflammation degrades the cap and inhibits resynthesis= dec in collagen = weak cap

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61
Q

Changes in BP and vasoconstriction can cause plaque rupture as well. if ruptured, they can form atherosclerotic debris (cholestrol emboli) causing?

A

partial or complete vascular occlusion when it reaches a vessel small enough

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62
Q

what is a localized abnormal dilation of a blood vessel or the heart that may be congenital or acquired?

A

Aneurysm

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63
Q

What is an intact but thinned muscular wall at the site of dilation, either saccular or fusiform?

A

True aneurysm

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64
Q

What is a defect through the wall of the vessel, communicating with an extravascular hematoma that freely communicates with the intravascular space… PULSATING HEMATOMA?

A

False aneurysm or pseudo-aneurysm

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65
Q

What arises when blood enters a defect in the arterial wall and tunnels between its layers, for example, between intima and media?

A

dissection

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66
Q

An aneurysm may occur whenever the connective tissue of the wall is weakened either acquired or congenital. What is an example of defective vascular wall CT? 2

A 
Marfan syndrome (defective fibrillin synthesis)
Ehler danlos
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67
Q

Aneurysms may also occur due to degradation of vascular wall CT via inflammatory conditions such as atherosclerosis leading to increase MMP. What are three ways the vascular wall is weakened by ischemia?

A

atherosclerosis (ischemia inner media)
HTN (ischemia of outer media)
** Tertiary Syphillis (ischmeia of outer media - thoracic aorta) **

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68
Q

Obliterative endarteritis (late stage syphillis) shows a predilection for small vessels including the vasa vasorum of the thoracic aorta. This leads to ischemic injury of the aorta media and?

A

aneurysmal dilation which involves the aortic valve annulus == aortic valve regurgitation

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69
Q

loss of vascular wall elastic tissue or ineffective elastin synthesis leads to what?

A

cystic medial degeneration (disorganized elastin, inc. ground substance)

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70
Q

cystic medial degeneration is a final common result of different conditions including ischemic medial damage and?

A

marfans syndrome

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71
Q

What are the two most important causes of aortic aneurysms?

A

athersclerosis (AAA) and hypertension (ascending aortic)

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72
Q

What can originate from septic emboli (d/t infective endocarditis), and extension of a suppurative process or circulating organisms directly infecting the arterial wall?

A

Mycotic aneurysms

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73
Q

What can be seen in cystic medial degeneration?

A

elastin fragmentation and areas devoid of elastin that resemble cystic spaces but are actually fillled with proteoglycans

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74
Q

AAA is due to athersclerosis in the abdomen below the renal arteries before the bifurcation, and often involve the iliac arteries. More frequent in? 3

A

men
smokers
6th decade of life

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75
Q

AAA are characterized by severe athersclerosis covered with mural thrombi. Sometimes detected as a pulsating mass and bruit. Complications include occlusion of arteries and downstream ischemia and was else? 3

A

rupture and hemorrhage
embolism
impingement of another structure

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76
Q

If a younger patient presented with back pain and elevated inflammatory markers (CRP) what can one expect?

A

Inflammatory AAA (5-10%)

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77
Q

Rupture risk of AAA is related to the size. aneurysms over 5cm are managed surgically. What percent rupture at 6cm?

A

25%

78
Q

Thoracic aortic aneurysm often due to hypertension, and sometimes marfans. Marfans is AD genetic DO with defective FBN1 gene, fibrillin protein that leads to?

A

abberant TGF-B activity that weakens elastic tissue

79
Q

Clinical presentation of thoracic aortic aneurysm include: respiratory difficulties, cant swallow d/t compression esophagus, persistent cough d/t compression laryngeal N, and what 2 other things?

A

Bone pain d/t erosin

aortic valve insufficiency; myocardial ischemia or valve rupture (most commonly seen in syphillis)

80
Q

Aortic dissections occur in hypertensive males aged 40-60 and younger patients with marfans. The primary risk factor is?

A

Hypertension

81
Q

The classic presentation of an aortic dissection is sudden onset of severe chest pain (begin anterior chest), and radiates to what?

A

radiates to the back between the scapulae and moves downward as the dissection progresses. (often confused with acute MI)

82
Q

Blood enters wall via an intimal tear forming an intramural hematoma. usually in hypertensive patients there is some degree of?

A

cystic medial degeneration

83
Q

Most dissections arise in the ascending aorta, 10cm from the aortic valve. When the dissections rupture through the adventitia what two things might occur?

A

massive hemorrhage in thoracic/abdominal cavity

Cardiac tamponade in pericardial sac

84
Q

If the dissecting hematoma reenters the lumen of the aorta through a second intimal tear it is called?

A

a new false vascular channel or double barreled aorta (can be endothelialized to become chronic dissections)

85
Q

Type A (proximal) involves the ascending aorta, either as a part of a more extensive dissection-entire aorta (DeBakey I) or in isolation- only ascending aorta (Debakey II). What can be seen in a Type B/DeBakey III?

A

dissections arise after the take-off of the great vessels (subclavian)

**Type A is the most common and dangerous

86
Q

Vasculitis is the term for vessel wall inflammation. Clinical presentation depends on the vascualr bed affected (CNS, heart, bowels). What are constitutional symptoms?

A

fever
malaise
arthralgia
myalgia

87
Q

Any type of vessel can be affected by vascultitis, but more commonly arterioles, capillaries and venules. What are the 2 types of vasculitis?

A

Noninfectious and infectious

88
Q

The major cause of noninfectious vasculitis is a local or systemic immune response. Immunologic injury may be cause by: immune complex deposition, antineutrophil cytoplasmic abs, antiendothelial cell bodies and?

A

autoreactive T cells

89
Q

What form of vasculitis is the only one involved with the aorta, and may have a clinical history of >40 y/o with polymyalgia rheumatica?

A

Giant Cell Arteritis

90
Q

What form of vasculitis requires granulomas?

A

granulomatosis with polyangitis (Wegener granulomatosis)

91
Q

What form of vasculitis requries eosinophils and may have a clinical history of asthma and atopy?

A

Churg-Strauss Syndrome

92
Q

What form of vasculitis ‘requires’ thrombosis and has a clinical history of a young male smoker?

A

Buerger Disease

93
Q

What form of vasculitis ‘requires’ neutrophils and has a clinic history of orogenital ulcers?

A

Behcet Disease

94
Q

Immune complex associated vasculitis is due to autoantibody production and formation of immune complexes which deposit in vascular walls, causing an inflammatory reaction. What are three scenarios where this can be seen?

A
  1. SLE (systemic immunologic dz)
  2. Drug Hypersensitivity
  3. Secondary to infectious agents
95
Q

Always consider drug sensitivity for immune complex vasculitis; PCN/streptokinase acts as a hapten binding to serum proteins of vessel walls. Can be mild to fatal with what being most common?

A

Skin lesions

96
Q

What is a heterogenous group of antibodies that react with cytoplasmic enzymes found in neutrophil granules, monocytes, and endothelial cells?

A

Antineutrophil cytoplasmic antibodies (ANCA) Non-infectious**

97
Q

There are two important ANCAs including PR3-ANCA (anti proteinase3) which is a neutrophil azurophilic granule associated with polyangitis, and MPO-ANCA (anti-myeloperoxidase) which is a lysosomal granule induce by rx propylthiouracil, and is associated with? (2)

A

microscopic polyangitis and churg-strauss syndrome

98
Q

ANCA titers generally follow disease severity, meaning a rise in titres predicts disease reoccurance. ANCA activate neutrophils, which then release?

A

reactive oxygen species

99
Q

ANCA are described as what? due to ANCA Ab are directed against cellular constituents and do NOT form circulating immune complexes?

A

pauci-immune

100
Q

Vascular lesions associated with ANCA usually do not contain?

A

Ab and complement complexes

101
Q

Giant cell (temporal) arteritis (non-infectious) is the most common form of vasculitis among older patients. It is a chronic (T cell mediated) inflammatory disorder of large to small-sized arterties that principally affect?

A

arteries in the head temporal but also the vertebral and opthalmic arteries

102
Q

Giant cell arteritis arteries develop intimal thickening and medial granulomatous inflammation with multinucleated giant cells and ?

A

fragmentation of the elastic lamina

103
Q

The healed sites of inflammation from giant cell arteritis shows scarring of the media and intimal thickening. Patients have constitutional symptoms (fever/fatigue/wt loss) as well as what other 3?

A

Facial pain near superficial temporal artery
Headache
**DOUBLE VISION- opthalmic A. — permanent blindness — medical emergency

104
Q

What is a noninfectious granulomatous vasculitis of medium and larger arteries characterized by ocular disturbances and marked weakening of the pulses in the upper extremities?

A

Takayasu Arteritis (pulseless disease)

105
Q

How is the distinction between giant cell temporal aortitis and takayasu aortitis made?

A

By age!
younger than 50 = takyasu
Older than 50= giant cell

106
Q

Symptoms of takayasu arteritis include a weak pulse and low blood pressure in the UE**, visual deficits, retinal hemorrhage, and what else?

A

dec BP in carotid, claudication of the legs

107
Q

takayasu has similiar histological findings as giant cell arteritis except it involve the aortic arch and major branch vessels and what other vessels may be involved? 3

A 
pulmonary A (leading to pulmonary HTN)
Coronary and renal As(lead to MI and systemic HTN)
108
Q

What is a systemic noninfectious vasculitis of small or medium sized muscular arteries, typically involving renal (heart, liver) and visceral vessels but sparing the pulmonary circulation?

A

Polyarteritis Nodosa (PAN)

109
Q

PAN may affect any age group, but classically young adult. Almost 1/3 have chronic hepatitis B and have what which are found in the vessels?

A

HBsAg-HBsAb immune complexes

110
Q

PAN is associated with segmental transmural necrotizing inflammation (neutrophils, eosinophils, lymphocytes and mø) and have a predilection for branch points. What are the first signs of dz? 3

A

ulcerations
infarcts
ischemic atrophy/hemorrhage

111
Q

PAN sites of inflammation are usually not cicumferential (one sided). An inflammed wall is susceptible to thrombus formation/occlusion, aneurysm and?

A

rupture

112
Q

Sx of polyarteritis nodosa result from ischemia and infarction of tissues/organs and vary. “Classic Presentation” can involve rapidly accelerating HTN due to renal A involvement, and what other 2?

A 
diffuse myalgias
peripheral neuritis (motor nerves)
113
Q

What is the major cause of mortality with PAN?

A

renal involvement (untreated PAN is fatal- immunosuppression cures 90%)

114
Q

Histologically in PAN, only half of the vessel will be involved, with thickened vessel wall to adventitia and what important thing?

A

fibrinoid necrosis**

115
Q

What is an acute febrile, usually self limited illness of infancy and childhood (80% of patients are 4y old or young), which is associated with a noninfectious arteritis affecting large and medium sized, even small vessels?

A

Kawasaki Disease

116
Q

Noninfectious kawasaki disease is mostly seen in children younger than 4. It often involves the coronary arteries which may form aneurysms causing what?

A

thrombosis or rupture and acutre MI at age 4!

117
Q

Clinically kawasaki presents with erythema of the conjuctiva, oral mucosa, and palms and soles. What other two sx do they present with?

A

desquamative rash and cervical LN enlargement-mucocutaneous LN syndrome (20% untreated will have coronary A problems in the future)

118
Q

What type of noninfectious necrotizing vasculitis generally effects capillaries, as well as small arterioles and venules, also called hypersensistivity or leukocytic vasculitis?

A

Microscopic polyangitis

119
Q

Unlike PAN, all lesions of microscopic polyangitis tend to be of the same age and are distributed more widely. It affects vessels of many organ systems, what two being the most common?

A

necrotizing glomeruloneprhitis (90%) and pulmonary capillaritis

120
Q

Most cases of microscopic polyangitis are associated with that?

A

MPO-ANCA

121
Q

Microscopic angiitis can be a feature of immune disorders such as henoch schoneein purpura, essential mixed cryoglobulinemia and vasculitis assoc. with CT disorders. What is the morphology characterized by?

A

segmental fibrinoid necrosis of the media and focal transmural necrotizing lesions, without granulomatous inflammation

**many apoptotic (fragmented) neutrophils seen

122
Q

The main sx of microscopic angiitis include hemoptysis, hematuria and proteinuria, bowel pain and what other two?

A

muscle pain/weakness and PALPABLE cutaneous purpura

123
Q

What is the leading cause of acquired heart disease in kids?

A

Kawasaki Disease

124
Q

What is a small vessel noninfectious nectrotizing vasculaitis classically associated with asthma, allergic rhinitis, lung infiltrates, peripheral hypereosinophilia, and extravascular necrotizing granulomata?

A

Churg-Strauss Syndrome

125
Q

Churg strauss is histologically similar to PAN or microscopic angiitis but are ALSO characteristically accompanied by what? (2)

A

granulomas and eosinophils**

126
Q

Less than 1/2 churg strauss show ANCA positivity (MPOANCA) and many organ systems may be involved, with cutaneous involvment palpable purpura, GI tract bleeding and?

A

renal disease (focal and segemental glomerulosclerosis)

127
Q

Churg Strauss has myocardial involvement which may give rise to cardiomyopathy, which is seen in 60% of patients and accounts for?

A

almost 1/2 of deaths in the syndrome

128
Q

What is a small to medium vessel neutrophilic vasculitis that classically presents as a clinical triad of recurrent oral ulcers, gential ulcers and uveitis (eye inflam)?

A

Bechet Disease

129
Q

Bechet dz can also involve Gi and pulmonary manifestations. The vessel inflammation is neutrophilic and morphologically nonspecific ; may involve visceral organ systems with aneurysm formation.. What gene is associated with this dz?

A

HLA-B51

130
Q

Bechet diesase mortality is related to sever neurologic involvement or?

A

rupture of aneurysms

131
Q

What has two names, and is a noninfectious vasculitis characterized by necrotizing granulomas, necrotizing or granulomatous vasculitis and focal necrotizing crescentic glomerulonephritis?

A

Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)

132
Q

Granulomatosis w polyangiitis is a form of T cell mediated hypersensitivity response to normall innocuous inhaled microbial or other environmental agents. what ANCA are 95% of cases associatd with?

A

PR3 ANCA

133
Q

Males are more commonly affected than females with granulomatosis w polyangiitis, with the average age being 40. Symptoms include pneumonitis and SINUSITIS and what else? 2

A

renal disease
nasopharyngeal ulceration

(rashes/myalgias less imp)

134
Q

If granulomatosis polyangiitis is left untreated, rapidly fatal with 80% moralitiy at one year. Seen chronic relapsing and remitting. Describe the morphology seen with this disease.

A

Granulomas with geographic patterns of central necorisis and vasculitis (upper resp)
necrotizing granulomas which may coalesce and cavitate (lower resp) imp like TB

135
Q

What is characterized by segmental, thrombosing, acute and chronic inflammation of medium and small sized arteries, principally the tibial and radial arteries with occasional secondary extension into the veins and nerves of extremities?

A

Thromboangiitis obliterans (Buerger disease)

136
Q

Buerger dz/thromboagiitis obliterand leads to vascular insufficiency of the extremities in smokers under the age of 35 with an increased prevelance in what ethnic groups? 3

A

israeli
indian subcontinent
japanese

137
Q

People with buerger dz hav hypersenitivity to intrademally injected tobacco products. Morphologically, acute and chronic inflammation accompanied by luminal thrombosis can be seen. Thrombus can contain small?

A

microabscesses compose of neutrophils surrounds by granulomatous inflammation

138
Q

Sympotms of thrombangiitis obliterans (buerger) include early manifestations of raynaud phenominon, leg pain induced by exercise. Vasc insuficiency can lead to what unique entity?

A

severe pain even at rest due to neural involvement (note: fingers/toes fall off to frank gangrene)

139
Q

What are the three vascular invasive agents that cause infectious vasculitis?

A

Pseudomonas (bact)
Aspergillus (septate hyphae)
Mucor (both fungi)

140
Q

Vascular infections weaken the arterial walls causing infectious mycotic aneurysms or can induce thrombosis or infarction. What can inflammation induced thrombosis of meningeal vessels in bacterial meningitis eventually cause?

A

infarction of the underlying brain tissue

141
Q

What is excessive vasospasm of small arteries and arterioles, especially finers and toes. Red white and blue stands for what?

A

proximal vasodilation, central vasconstriction and distal cyanosis

142
Q

Primary raynaud phenomenon (raynaud dz) is induced by cold or emotions and is benign. Who is more apt to have it and what kind?

A

3-5% of population ; young women

SYMMERIC involvement of the digits

143
Q

Secondary raynaud phenomenon is due to arterial disease such as SLE, scleroderma, thromboangiitis obliterans and will do what with time?

A

worsens with time as intimal thickening increases

*asymmetric involvement of digits

144
Q

What is excessive vasoconstriction of myocardial arteries or arterioles (cardiac raynaud) which may cause ischemia or infarct?

A

Myocardial vessel vasospasm

145
Q

High levels of vasoactive mediators can precipitate prolonged myocardial vessel contraction, caused by what endogenous and exogenous vasoactive agents?

A

epinephrine, pheochromocytoma

cocaine

146
Q

Greater than 20 minutes of vasoconstriction in a coronary artery can lead to?

A

myocardial infarction

147
Q

Two outcomes of myocardial vessel vasospasm is sudden cardiac death or what? whicih is called broken heart syndrome due to emotional duress

A

Takotsubo cardiomyopathy

*may see contraction band necrosis

148
Q

What is an abnormal dilation of veins with valvular incompetence, secondary to sustained intraluminal pressure?

A

varicose veins (superficial veins)

149
Q

Incompetence of venous valves can lead to stasis, congestion, thrombus, edema, pain and ischemia of the overlying skin. What are two common complications?

A

poor wound healing

superimposed infections

150
Q

Embolism from thrombi of superficial lower extremity veins is RARE, as opposed to?

A

deep vein thrombosis DVT - they do embolize

151
Q

What are 3 risk factors of varicose veins?

A

obesity
pregnancy
genetics

152
Q

Portal hypertension due to cirrhosis opens porotsystemic shunts which direct blood to veins at the gastroesophageal junction causing?

A

esophageal varices (may rupture)

portal HTN also causes caput medusa (periumbilical veins)

153
Q

What is a dilation of the venous plexus at the anorectal junction which is extremely common and cause pain, bleeding and ulcers?

A

hemorrhoids

154
Q

Thrombophlebitis is venous thrombosis and inflammation. 90% involve the deep veins in the legs and can be asymptomatic. What is the single most important risk factor of DVT?

A

in LE, prolonged inactivity or immobilization

systemic hypercoagulability may also increase risk

155
Q

Migratory thrombophlebitis (trousseau sign) is seen in patients with cancer and hypercoagulability as a paraneoplastic syndrome. Also seen in what types od adenocarcinomas?

A

mucin-producing adenocarcinoma (mucin is thrombogenic) of the lung ovary pancreas

156
Q

Superior vena cava syndrome occurs when neoplasms compress or invade the SVC. What are 3 neoplasms that could do this?

A

bronchogenic carcinoma
mediastinal lymphoma
Aortic aneurysm

157
Q

Obstruction of the SVC producves a characteristic clinical complex including marked dilation of the veins of the head neck and arms with cyanosis as well as?

A

respiratory distress d/t pulmonary vessels being compressed

158
Q

IVC syndrome is neoplasms that compress or invade the IVC or thrombosis of the hepatic, renal or LE veins that propagates cephalad. What two neoplasms are known to occlude the IVC and right atrium?

A

hepatocellular carcinoma HCC

renal cell carcinoma RCC

159
Q

IVC obstruction induces marked LE edema and distention of the superficial collateral veins of the lower abdomen. If the kidney is involved, what is seen?

A

massive proteinuria

160
Q

Lymphangitis is acute inflammation and spread of bacterial infection into the lymphatics, most commonly caused by?

A

group A B hemolytic streptococci

161
Q

Lymphangitis is manifested by red painful subcutaneous streaks and painful enlargement of the draining LNs known as?

A

lymphadenitis

162
Q

Primary lymphadema is an isolated congential defect of familial Milroy disease. What is secondary lymphedema?

A

stems from bloackage of normal lymphatic,

163
Q

Secondary lymphedema can be caused by malignant tumors, axillary ln removal in radical mastectomy, postinflammatory thrombosis and scarring, and what other 2?

A

Postirradiation fibrosis

Filariasis

164
Q

What is seen in skin overlying breast cancer after the draining lymphatics are clogged with tumor cells?

A

peau’d orange (orange peel look)

165
Q

Chylous ascites (abdomen), chylothorax and chylopericardium is what that can be seen after rupture of dilated lympathic lets say secondary to obstruction from a tumor?

A

milky accumulations of lymph

166
Q

What kind of tumors produce obvious vascular channels filled with blood cells lined by a monolayer of normal appearing endothelial cells?

A

Benign vascular tumors

167
Q

What kind of tumors are more cellular and proliferative and exhibit atypia, and do not form well organized vessels- confirmed by markers such as CD31 and vWF?

A

Malignant vascular tumors

168
Q

What is a generic term for any local dilation of a structure, while telangiestasia is used to describe permanent dilation of preexisiting small vessels that form a red lesion?

A

ectasia

169
Q

what is the most common form of vascular ectasia, which is a light pink to deep purple flat lesion on the ehad or neck?

A

Nevus flammeus (birthmark)

  • regress spontaneoulsy
170
Q

Port wine stain is a form of nevus flammeus which grow during childhood. If in the trigeminal nerve area, can be associated with sturge weber syndrome. What is this syndrome associated with? 6

A 
facial port wine nevi
ipsi venous angiomas in cortical leptomeninges
mental retardation
seizures
hemiplegia
skull radio opacities
171
Q

what are nonneoplastic vascular lesions resembling a spider, which manifest as radial and often pulsatile arrays of dilated subcutaneous arties or arterioles abouta central core that blanch with pressure?

A

spider telangiestasias

172
Q

What is an AD DO caused by mutations in a gene that encodes TGFB, widely distributed over the skin and oral mucous membranes as well as in ? 3

A

respiratory, GI and urinary tracts

*lesions can rupture and cause bleeding

173
Q

What are common tumors with localized increase in neoplsatic blood vessels?

A

Hemangiomas

congenital/juvinile/strawberry HA regress

174
Q

What is the most common benign vascular tumor with thin wall capillaries that are tightly packed together?

A

Capillary hemangioma

175
Q

What type of HA is irregular, dilated vascular channels making a lesion with an indistinct border? More likely to involve deep tissue and to bleed…

A

Cavernous HA

see channels/caverns

176
Q

What is a type of capillary HA that is rapidly growing and often in oral mucosa following trauma?

A 
Pyogenic granuloma (it is not either)
aka lobular capillary HA
177
Q

What occurs during pregnancy in the gingiva of pregnant women? usually regress but sometimes need surgery to remove

A

granuloma gravidarum

178
Q

A simple lymphangioma appears very similar to capillary hemangiomas but without RBCs. which occur predominately in what 3 areas?

A

in the subcutaneous tissue of the head neck and axilla

179
Q

Cavernous lymphangioma aka cystic hygroma is found in the neck/axilla of children. Large cavernous lymphangiomas of the neck are seen in what syndrome?

A

Turner Syndrome (XO)

180
Q

What is a benign tumor arising from the glomus bodies of SM origin (HA from endothelial cells) and most often appear in the distal fingers? (PAINFUL)

A

Glomus tumors

181
Q

Bacillary angiomatosis is a vascular proliferation in response to gram negative?

A

bartonella bacilli (cat scratch dz)

182
Q

Bacillary angiomatosis only occurs in immunocompromised patients, lesions are red and localized. Proliferation of capillaries with plump endothelial cells. How can it be visualized and treated?

A

visualized using warthin starry stain

treated with macrolide abx

183
Q

What is neoplsatic endothelial cells are plump and cuboidal, resembling epithelium, with vascular channels being hard to recognize? (variable clinical behavior; 20-30% metastasize)

A

epitheliod hemagioendothelioma

184
Q

Kaposi Sarcoma is caused by human herpes virus 8 HHV8. What are the four different forms?

A

AIDS associated
Classic
Endemic African KS
Transplant associated

185
Q

AIDS associated KS is the most common form seen in the US and most common aids related tumor. It may spread to LN and viscera. What form is seen in older men from middle easter, mediterranean or eastern european decent not associated with HIV?

A

Classic ks (tumors localized to skin)

186
Q

Transplant assoc KS is not assoc with HIV but with T cell immunosuppression and can spread to the LN and viscera. What is seen in people under 40 involving the LNs?

A

Endemic African KS

187
Q

Kaposi Sarcoma can be seen as coalescent red-purple macules and plaques of skin. What can be seen histological in the nodular stage of KS?

A

sheets of plump, proliferating spindle cells

188
Q

Angiosarcoma is a malignant endothelial tumor seen in older men and women. It can be induce by?

A

radiation exposure and can arise in the setting of lympedema (after radical mastectomy)

189
Q

Hepatic angiosarcoma is associated wtih arsenic, pesticides, thorotrast, and polyvinyl chloride (PVC). What are the most common sites where it can be seen? (4)

A 
skin
soft tissue
breast
liver
(locally invasive/may metastasize/5yr survival 30%)
190
Q

Microscopically, angiosarcoma has all degress of differentiation from plump atypical endothelial cells forming vascular channels to wildly undifferentiated tumors with a solid spindled appearance and no discernible blood vessels that may be difficult to distinguish from carcinomas and melanomas. What two markers are used to determine its endothelial origin?

A

CD31 brown stain

or vWF

191
Q

What do drug-eluting stents do? *note: angioplasty and coronary stents usually done together 90% of the time

A

leach antiproliferative drugs to block SM activation, decreasing re-stenosis at 1yr by 50-80% (paciltaxel & sirolimus)

21. CPR Exam 1 (26 decks)

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