Vascular Flashcards
1
Q
Basic Principles: what are the 2 clinical features of vasculitis?
A
- Nonspecific symptoms of inflammation (fever, fatigue, weight loss, and myalgias) - Symptoms of organ ischemia (due to luminal narrowing or thrombosis of inflammed vessels)
2
Q
How are vasculitides subdivided? What does each entail?
A
- Large-vessel vasculitis (aorta + major branches) - Medium-vessel (muscular arteries that supply organs) - Small-vessel (arterioles, capillaries, and venules)
3
Q
What are the 2 Large-vessel vasculitides in pathoma?
A
- Temporal (Giant Cell) Arteritis - Takayasu Arteritis
4
Q
What type of vasculitis is Temporal (giant cell) Arteritis & what vasculature does it typically affect?
A
- It is a granulomatous vasculitis and involves branches of the carotid artery
5
Q
What type of patients (age and sex) are usually affected by Temporal giant cell arteritis?
A
Older adults (>50 years of age); this is the most common form of vasculitis in older adults. Females are usually affected.
6
Q
What flu-like symptoms w/ joint and muscle pain is assoc. with temporal giant cell arteritis?
A
Polymyalgia rheumatica
7
Q
What characteristic sign is seen with ESR in temporal giant cell arteritis?
A
ESR is usually elevated (> 100)
8
Q
What would biopsy reveal in Pts w/ temporal giant cell arteritis?
A
Inflammed vessel wall + giant cells (due to granulomas) w/ intimal fibrosis (due to healing response that follows)
9
Q
Why is a negative biopsy not sufficient to rule out large-vessel vasculitis?
A
The lesions are segmental = the artery may only be affected in 1 specific area –> if you get a negative biopsy, you may have simply missed the section that has the lesion.
10
Q
What is the treatment for temporal giant cell arteritis? When is it administered?
A
Corticosteroids; administer immediately/empirically even if diagnosis is not confirmed if you suspect pt having this disease.
11
Q
What is the biggest risk of delaying treatment in temporal giant cell arteritis?
A
Pt may develop irreversible blindness if thrombosis/fibrosis occurs in opthalmic artery (branch of carotid artery)
12
Q
What type of vasculitis is takayasu arteritis & what vasculature does it typically affect?
A
- Granulomatous vasculitis and involves aortic arch @ branch points.
13
Q
What type of patients (age, sex, race) are usually affected by Takayasu arteritis?
A
Adults
14
Q
What clinical signs make Takayasu arteritis also known as “pulseless disease”?
A
Pts will have weak or absent pulse in the upper extremities
15
Q
What is Rx for Takaysu arteritis?
A
Corticosteroids
16
Q
What are 3 clinical ways to differentiate Takayasu from Temporal Giant Cell Arteritis?
A
1) vasculature involved in Takayasu is more proximal (aortic arch) rather than distal as in Temporal (branches of Carotid Artery)
2) Pts affected by Takayasu are typically under 50 years of age (young adults); while Temporal patients are usually above age 50
3) Takayasu is also known as pulseless disease and presents w/ weak or absent pulse in UE; while Temporal is assoc w/ flu-like joint/muscle pains (polymyalgia rheumatica)
17
Q
What are the 3 medium-vessel vasculitides from pathoma?
A
- Polyarteritis nodosa
- Kowasaki disease
- Buerger Disease
18
Q
What type of vasculitis is polyarteritis nodosa and what does it usually affect?
A
Necrotizing vasculitis; involves multiple organs except lungs
19
Q
What are 4 clinical symptoms that are usually present in polyarteritis nodosa?
A
- HTN (due to renal artery involvement)
- Abdom pain + melena (due to mesenteric artery)
- Neuro disturbances
- Skin lesions
20
Q
What serum marker is assoc. w/ polyarteritis nodosa?
A
Serum HBsAg
21
Q
Why is polyarteritis nodosa referred to as having a “string of pearls” appearance on imaging?
A
Early lesions in polyarteritis nodosa is a transmural inflamm –> weakens bv wall –> aneurysms can form (pearls); this is followed by a segment of artery that has fibrosis (late lesion) –> the continuous pattern of early lesion aneurysm + late lesion fibrosis = string of pearls appearance
22
Q
What is the pathognomic finding of polyarteritis nodosa that is found on imaging?
A
string of pearls appearance
23
Q
What is the Rx for polyarteritis nodosa?
A
Corticosteroids + cyclophosphamide
24
Q
What type of patients (age and race) are classically affected by Kawasaki disease?
A
Asian children <4 years of age
25
What type of symptoms do pts w/ Kawasaki disease present with?
Non-specific findings that resemble viral infxn (erythematous rash on palms and soles, enlarged cervical ln, fever, and conjunctivitis)
26
What are 2 major complications to worry about in Kawasaki disease?
1. Thrombosis w/ MI
2. Aneurysm w/ rupture
27
What vasculature is most often affected by Kawasaki disease?
Coronary artery
28
What are the Rx's that are used in Kawasaki disease? Why is one of them controversial?
- ASA + IVIG are used in Kawasaki
- ASA should never be given to children \>4 years of age if they are suffering from viral infxn since it can precipitate Reye's Syndrome (encephalitis + liver failure); however, symptomology of Kawaski resembles that of viral infxn
29
What is the major risk factor for developing Buerger disease?
- Smoking
30
What type of vasculitis is Buerger syndrome? What does it normally affect?
Necrotizing vasculitis that involves digits
31
What is one classic clinical feature of Buerger disease that causes discoloration of digits?
Raynaud phenomenon (vasospasms that cause affected area to turn from white --\> blue --\> red)
32
What is the Rx for Buerger Disease?
Smoking cessation
33
What are the 4 small-vessel vasculitides according to Pathoma?
1. Wegener granulomatosis
2. Microscopic polyangiitis
3. Churg-Strauss Syndrome
4. Henoch-Schonlein Purpura
34
What type of vasculitis is Wegener granulomatosis? What areas does it normally affect?
- necrotizing granulomatous vasculitis
- nasopharynx, lungs, and kidneys (recall that wegener has a "C" shaped distribution of symptoms if you draw a stick-figure)
35
What is the classic presentation of Wegener granulomatosis (type of patient + 3 symptoms)
- middle age man w/:
1. sinusitis or nasopharyngeal ulceration
2. hemoptysis w/ bilateral nodular lung infiltrates
3. hematuria due to rapidly progressive glomerulonephritis (RPGN)
36
What is the hematuria seen in Wegners granulomatosis due to?
Rapidly progressive glomerulonephritis (RPGN)
37
What serum marker correlates with disaease activity in Wegner granulomatosis?
c-ANCA
38
What does biopsy show in Wegener granulomatosis?
Large necrotizing granulomas w/ adj necrotizing vasculitis
39
What is the Rx for wegener granulomatosis?
- Cyclophosphamide + steroids
40
What type of vasculitis is Microscopic Polyangiitis? What areas of the body does it affect?
- Necrotizing vasculitis
- Multiple organs, esp lung and kidney
41
How can you tell microscopic polyangiitis apart from wegener granulomatosis clinically and histologically?
- Microscopic polyangitis does not present with nasopharyngeal ulcers and do not have granulomas
42
What serum marker correlates with disease activity of microscopic polyangiitis?
p-ANCA
43
What is the Rx for microscopic polyangiitis?
Corticosteroids + cyclophosphamide
44
What type of vasculitis is Churg-Strauss Syndrome? What organs does it especially involve?
- Necrotizing granulomatous inflamation
- Lungs and heart
45
What are 2 classic clinical presenetations found in Pts who have Churg-Strauss syndrome?
1. Asthma
2. Peripheral eosinophilia
46
What serum marker correlates w/ disease activity in Churg-Strauss Syndrome?
p-ANCA
47
What are 2 ways you can tell Churg-Strauss apart from Microscopic Polyangiitis?
1. Churg has granulomas, MP does not
2. Churg has asthma + peripheral eosinophilia, MP does not
48
What is the most common vasculitis in children?
Henoch-Shonlein Purpura
49
What is the underlying cause of symptoms in Henoch-Shonlein Purpura?
IgA immune complex deposition
50
What are 3 classic symptoms found in Henoch-Shonlein Purpura?
1. Palpable purpura on buttocks and legs
2. GI pain and bleeding
3. Hematuria (IgA nephropathy)
51
What causes the hematuria found in Henoch-Shonlein Purpura?
IgA deposition in mesangium of glomerular capillaries
52
What kind of disease precipitates Henoch-Shonlein Purpura? Why?
- Henoch-Shonlein Purpura usually occurs following an upper respiratory infxn
- This is b/c IgAs form during URIs in order to protect mucosal sites --\> Pts w/ Henoch-Shonlein Purpura would produce too much and it would then deposit into vasculature
53
What numerical value is Systemic HTN defined as?
Pressure \> 140/90 mmHg (normal is \< 120/80 mmHg)
54
What are 2 ways HTN can be subdivided?
1. Primary vs Secondary HTN
2. Benign vs Malignant HTN
55
What are the most common forms of HTN?
Primary and Benign HTN are the most common forms of HTN
56
What is the etiology of primary HTN? What are 3 risk factors assoc. with it?
- Unkown etiology in 95% of cases
- Genetic factors; High Angiotensin II; Small # of nephrons (either congential, from injury, or genetic)
57
What are 5 modifiable risk factors assoc with HTN?
"Real HALOS" = Reduced physical activity; High sodium intake; Alcohol consumption; Low potassium intake; Smoking
58
What are the 4 steps by which renal artery stenosis causes secondary HTN?
1. Stenosis decreases blood flow to glomerulus
2. JGA responds by secreting renin, which converts AT to AT I
3. AT I is converted to AT II by ACE in the lung
4. ATII raises BP
59
How does ATII raise BP?
1. ATII contracts arteriolar smooth muscle, thereby increasing TPR
2. ATII promotes adrenal release of Aldosterone = increases sodium resorption in DCT in exchange for potassium = expands plasma volume
60
What are 2 characteristic findings of secondary HTN caused by renal artery stenosis?
1. Increased plasma renin
2. Unilateral atrophy of affected kidney (due to low blood flow)
61
What are two important causes of stenosis. Which gender and age group is assoc with each cause?
1. Atherosclerosis and Fibromuscular Dysplasia
2. Atherosclerosis is assoc w/ elderly men; Fibromuscular Dysplasia is assoc w/ young females
* Side note: Fibromusclar dysplasia is a developmental defect of bv wall that causes irregular thickening of large & medium-sized arteries (esp. renal artery)*
62
What are clinical manifestations of benign HTN?
None; benign HTN is clinically silent and damages vessels and organs slowly over time
63
What is malignant HTN and what is it defined as numerically?
Malignant HTN is a severe elevation in BP ( \>180/120 mmHg)
64
How do you treat malignant HTN?
IV Sodium Nitroprusside (NO-releasing drug)
65
What is a DASH diet? What does it include and emphasize?
DASH = Dietary Approaches to Stop HTN
- Includes low Na+, low fat, low caffeine, low Et-OH
- Emphasizes whole grains, fruits, veggies, limited Et-OH
66
What are the 3 arterioscleroses in Pathoma? What kind of blood vessels do they each affect?
1. Atherosclerosis (M/L-sized vessels)
2. Arteriolosclerosis (small bv's)
3. Monckeberg Medial Calcific Sclerosis (medium bv's)
67
What layer of vessel wall does atherosclerosis occur? What does it consist of?
- Intimal layer
- Necrotic lipid core (mostly cholesterol) + fibromuscular cap
68
What are the 4 major blood vessels that are commonly affected by atherosclerosis?
1. abdominal aorta
2. coronary artery
3. popliteal artery
4. internal carotid artery
69
What are the 2 subdivisions of risk factors for atherosclerosis? What does each entail?
- Modifiable and Nonmodifiable risk factors
- Modifiable risk factors: HTN, hypercholesterolemia, smoking, and diabetes
- Nonmodifiable risk factors: age (# and severity of lesions increase w/ age), gender (increased risk in males and postmenopausal females; estrogen is protective), and genetics (family hx is highly predictive of risk)
70
What are the 3 steps in pathogenesis for atherosclerosis development?
1. Dmg to endothelium allows lipids to leak into intima
2. lipids are oxidized and consumed by macrophages --\> produce foam cells that collect to produce fatty streaks
3. Inflammation and healing leads to ECM deposition and proliferation of smooth muscle (forming fibromuscular cap + necrotic lipid core)
71
What is the early lesion of atherosclerosis? What does it consist of? How early can it arise?
- Fatty streak (flat yellow lesions of the intima)
- lipid-filled macrophages
- Can be seen in most teenagers
72
What are 4 major complications of atheroscleosis?
1. Stenosis of medium-sized vessels
2. Plaque rupture w/ thrombosis
3. Plaque reupture w/ embolization
4. Weakening of vessel wall
73
Stenosis of medium-sized vessels due to atherosclerosis can lead to what 3 results? What vessels would be responsible?
1. Peripheral vascular disease (lower extremity arteries - i.e. popliteal)
2. Angina (coronary arteries)
3. Ischemic bowel disease (mesenteric arteries)
74
How much atherosclerotic stenosis would it take before complications arise?
Complications usually don't start until \>70% of lumen becomes stenotic
75
What are 2 results of plaque rupture w/ thrombosis due to atherosclerosis? What vessels would be affected?
1. Myocardial Infarction (coronary artery)
2. Stroke (Middle cerebral artery)
76
What is characteristic of atherosclerotic emboli?
Cholesterol crystals/clefts
77
What can result from weakening of vessel wall due to atherosclerosis? Where is it most commonly found?
Aneurysm; most commonly found in abdominal aorta before bifurcation but after renal artery branches
78
Why does atherosclerosis weaken vessel walls?
Atherosclerosis produces a diffusion barrier that prevents O2 from diffusing and nourishing the wall layers --\> bv wall becomes weak and atrophic
79
What are the two types of arteriolosclerosis? What is the cause of each of them?
- Hyaline arteriolosclerosis: endothelial dmg allows proteins to leak into the vessel wall, thereby causing vascular thickening
- Hyperplastic arteriolosclerosis: thickening of vessel wall due to hyperplasia of smooth muscle layer
80
What are the two risk factors of hyaline arteriolosclerosis? How does each one contribute to hyaline arteriolosclerosis development?
- long-standing benign HTN: increased BP will cause hydrostatic pressure to push proteins out
- diabetes: pt's w/ diabetes get non-enzymatic glycosylation of basement membrane --\> dmges vessel wall and causes it to be leaky --\> protein can then leak out into the vessel wall
81
What is a classic clinical result of hyaline arteriolosclerosis?
- glomerular scarring (arteriolonephrosclerosis) which slowly progresses to chronic renal failure
82
Which arteriosclerosis is described as having an "onion-skin appearance"?
Hyperplastic arteriolosclerosis
83
What disease precipitates hyperplastic arteriolosclerosis?
Malignant HTN
84
What causes the "flea-bitten" appearance associated with hyperplastic arteriolosclerosis?
Fibrinoid necrosis of vessel wall w/ hemorrhage
85
What is a classic clinical outcome of persistent hyperplastic arteriolosclerosis?
Acute renal failure
86
What vessel layer does Monckeberg Medial Calcific Sclerosis affect? What dpes it do and what type of arteries are affected?
- Medial layer
- Calcification of the media of muscular (medium-sized) arteries
87
What are clinical complications assoc w/ Monckeberg Medial Calcific Sclerosis?
It is clinically insignificant since it is not obstructive; it is usually found incidentally on x-ray or mammography
88
What happens in aortic dissection?
Blood flows through the intima of a bv wall due to some dmg --\> dissects through the medial layer, thereby causing a dissection of the original bv wall
89
What is the most common site for aortic dissection (specifically)? Why?
Proximal 10cm of aorta; it is a high stress region
90
What are 2 requirements for an aortic dissection to occur?
1. Aortic dissection occurs @ proximal 10cm of aorta (high stress region)
2. There must be pre-existing weakness of the media
91
What is the most common cause of aortic dissection in younger individuals and what is the most common cause of AD in older adults?
1. Inherited defects of connective tissue (young adults) - such as Marfan and EDS
2. HTN (older adults)
92
How does HTN predispose older adults to aortic dissection?
HTN causes hyaline arteriolosclerosis of the vaso vasorum (bv that supplies blood to outer wall layers of aorta) --\> decreased flow/nourishment casues atrophy of the media = weakens vessel wall
93
What are 2 inherited defects of connective tissue assoc w/ aortic dissections in younger individuals?
Marfan Syndrome (defective fibrillin) & Ehlers-Danlos Syndrome (defective collagen)
94
What is the classic symptom of aortic dissection that patients describe?
Sharp, tearing chest pain that radiates to the back
95
What are 3 complications of aortic dissection?
1. Pericardial tamponade (most common cause of death)
2. Rupture w/ fatal hemorrhage
3. Obstruction of branching arteries, which results in end-organ ischemia
96
What is the classic underlying disease that causes thoracic aneurysms? What is the mechanism of this process?
Tertiary syphilis; Endarteritis (inflamm of small bv; causes a "tree-bark appearance) of the vaso vasorum --\> luminal narrowing -\> decreases flow --\> atrophy of the aoric vessel wall
97
What is the major complication assoc w/ thoracic aneurysms?
Dilation of aortic valve root --\> aortic insufficiency (aorta can't pump out enough blood since it regurgitates back through the weakened valve)
98
Where do abdominal aortic aneurysms typically occur (specificially)?
Below the renal arteries, but above the aortic bifurcation into common illiacs
99
What are abdominal aortic aneurysms primarily caused by? What is the classic patient population for this disease?
- Primarily caused by atherosclerosis
- Classically seen in male smokers \> 60 years old w/ HTN
100
How do abdominal aoric aneurysms present clinically?
Presents as a pulsatile abdominal mass that grows w/ time
101
What is the major complicaiton of abdominal aortic aneurysms?
Major complication is rupture, especially when \> 5cm in diameter
102
What is the classic clinical triad of a ruptured abdominal aortic aneurysm?
Hypotension + pulsatile abdominal mass + flank pain
103
What are the 3 vascular tumors in pathoma?
1. Hemangioma
2. Angiosarcoma
3. Kaposi Sarcoma
104
What is a hemangioma?
Benign tumor made up of blood vessels
105
When do hemangiomas typically occur? What is the treatment?
- Hemangiomas typically present @ birth
- Often regresses on its own during childhood
106
What areas of the body do hemangiomas typically affect?
Skin and liver
107
What is an angiosarcoma?
Malignant proliferation of endothelial cells (but not complete blood vessels)
108
How can you differentiate between a hemangioma and purpura? What is the basis for this test?
- If you press on the skin lesion, it will turn pale if it is a hemangioma but would not change color if it was purpura
- Hemangiomas are tumors of blood vessels --\> if you press on a hemangioma, you will displace the blood out of the vessel, thereby causing it turn pale. However, purpura is blood that is in the skin, and therefore would not be displaced by pressing on it
109
Is there a change in appearance if you press on a lesion of Kaposi Sarcoma? Why?
No, because Kaposi is a proliferation of endothelial cells and not complete blood vessels --\> blood would not be displaced (as in hemangiomas), as it is not in a vascular channel but rather is intersperesed b/w the proliferated endothelial cells
110
What are common sites for angiosarcomas?
Skin, liver, and breast
111
What are 3 environmental exposure-related causes of liver angiosarcomas?
1. polyvinyl chloride (PVC)
2. arsenic
3. Thorotrast (a radiocontrast that was used back in the 1930s/40s)
112
What infxous cause is assoc w/ Kaposi Sarcoma?
HHV8
113
How does Kaposi Sarcoma present clinically? What areas of the body are affected?
Purple patches, plaques, and nodules on the skin; may also involve visercal organs
114
What are 3 classic patient populations that are affected by Kaposi Sarcoma? How would you treat each of them?
1. Older Eastern European males - surgical removal of tumor (they usually remain localized to skin)
2. AIDS - antiretroviral agents for AIDS --\> boost immune system to clear out the HHV-8
3. Transplant recipients - decrease immunosuppression --\> boost immune system to clear out HHV-8
115
What is the most common type of cerebral aneurysm? What is the pathological cause of it?
- Berry aneurysm
- Congenital defect in smooth muscle distribution @ branch points of the arterial wall (hence it is mostly found b/w ant. communicating and ant. cerebral arteries in Circle of Willis)
116
Following coronary bypass surgery, what is the most frequent cause of saphenous graft failure several years later?
Restenosis due to atherosclerosis
117
How does Behcet Syndrome present clinically?
Recurrent aphthous ulcers + genital ulcer + uveitis
118
What is the etiology of Behcet Syndrome? What other disease process is it associated with?
- Immune complex vasculitis of small vessels
- Can be seen after viral infxn
119
What disease presents as a tumor-like lesion consisting of large vascular channels that are interspersed w/ small capillary-type vessels?
Cavernous hemangioma
120
Where do cavernous hemangiomas most frqeuently present clinically (what organ)? What is colloquial term used to describe the lesion?
- Skin
- Port-wine stains
121
How can cavernous hemangiomas cause neurologic symptoms?
The hemangiomas may also occur in the brain (although less frequently than skin) --\> after a long quiescent period, it will slowly enlarge and cause neurologic symptoms
122
Where are glomus bodies (aka glomus apparatus) found? What do they do?
- Glomus bodies are peripheral receptors found in dermal layer of the skin
- They are sensitive to temperature and are involved in regulating body temperature through arteriolar blood flow
123
How would glomus tumors present clinically?
Painful, raised, red lesion on the skin
124
A histological biopsy of a glomus tumor would reveal what findings?
2 main histologic components of glomus tumor are:
1. Brancing vascular channels in connective tissue stroma
2. Aggregates of specialized glomus cells (nests of round regular cells)
125
How does Kaposi Sarcoma present dermatologically? What is the causitive agent of the disease?
- Multiple purple-colored skin nodules on hands and feet
- HHV8
126
What would skin biopsy reveal in a Pt w/ Kaposi Sarcoma?
Numerous poorly differentiated spindle-shaped neoplastic cells and vascular lesions filled w/ RBCs
127
What is endocardial fibroelastosis and what patient population is most often affected?
Rare heart disorder usually occuring in children 2 years or younger, where Pt develops unexplained heart failure due to increased amt of supporting CT and elastic fibers
128
What is the primary cause of restrictive cardiomyopathy in children?
Endocardial fibroelastosis
129
What would be seen histologically in an aortic dissection? What is this process called?
- Focal loss of elastic and muscle fibers in the aortic media --\> this leads to cystic spaces that get filled w/ metachromatic myxoid material
- Cystic Medial Necrosis
130
What can trigger Raynaud's Phenomenon to happen in a Pt who is susceptible to it (as in what can increase the frequency by which it happens)?
Cold temperature and emotional stimuli can precipitate Raynaud's Phenomenon
131
What are varicose veins? What dermatologic condition can this cause if not treated?
- An enlarged and tortuous blood vessel (usually occurs due to our upright posture --\> blood flow becomes slow and leaks from engorged capillaries)
- Stasis dermatitis
132
What are hemorrhoids?
Dilations of veins of the rectum and anal canal, which may occur inside or outside the anal sphincter
133
What is the principal cause of vasculopathy in Pts w/ scleroderma?
Subintimal fibromsuclar thickening --\> luminal narrowing
134
