CHAPTER 7: VASCULAR PATHOLOGY Flashcards Preview

Pathoma > CHAPTER 7: VASCULAR PATHOLOGY > Flashcards

Flashcards in CHAPTER 7: VASCULAR PATHOLOGY Deck (127):
1

What is the definition of vasculitis?

Inflammation of the blood vessel wall

2

What are the three layers of an artery?

Intima= endothelial
Media= muscle
Adventitia= connective tissue

3

What is the etiology of vasculitis?

Unknown--most are NOT INFECTIOUS

4

Generally, what are the symptoms associated with vasculitis?

1) Non-specific sx. of inflammation (fever, fatigue, weight loss, myalgia)
2) Sx. of ORGAN SPECIFIC ischemia

5

What causes the organ specific ischemia seen in vasculitis?

- Luminal narrowing
- Thrombosis of inflamed vessels

6

What does large vessel vasculitis involve?

Aorta and its major branches

7

What does medium vessel vasculitis involve?

Muscular arteries and organs supplied by those arteries

8

What does small vessel vasculitis involve?

Arterioles, capillaries, venules

9

What type of vasculitis is Temproal (Giant Cell) Arteritis?

Large-vessel

10

What is Temporal Arteritis?

Granulomatous vasculitis that involves the BRANCHES OF THE CAROTID ARTERY
- Temporal a.
- Ophthalmic a.

11

What is the typical patient affected by Temporal Arteritis?

- Older adult (greater than 50 y/o)
- Female

12

How does Temporal Arteritis present?

1) Headache= temporal a. involvement
2) Visual disturbances= ophthalmic a. involvement
3) Jaw claudication

+Flu-like sx.

13

What lab is often elevated with Temporal Arteritis?

ESR

14

What will biopsy reveal in Temporal Arteritis?

- Inflamed vessel wall w/ Giant Cell (muli-nucleated large cells)
- Lesions are SEGMENTAL

****B/c lesions are segmental, must biopsy a large segment AND negative biopsy does NOT r/o disease*****

15

How is Temporal Arteritis treated?

Corticosteroids

16

Why is prompt treatment of Temporal Arteritis crucial?

Untreated there is a high risk of blindness

17

What is Takayasu Arteritis?

Granulomatous vasculitis that involves the AORTIC ARCH at branch points

18

How does Takayasu Arteritis clinically differ from Temporal Arteritis?

1) Patients are LESS than 50 y/o
2) Asian

19

How does Takayasu Arteritis present?

Young asian female with:
1) Visual disturbances/ neurologic sx.
2) Weak/ absent upper extremity pulses

20

What clinical aspects of Takayasu Arteritis and Temporal Arteritis are shared?

- Treatment= corticosteroids for both
- ESR elevation is common to both

21

What type of vasculitis is Polyarteritis Nodosa?

Medium vessel

22

What is Polyarteritis Nodosa?

Necrotizing vasculitis involving multiple organs, BUT NOT THE LUNGS

23

How does Polyarteritis Nodosa classically present?

Young adult with:
1) HTN (renal a. involvement)
2) Abdominal pain and melena (mesenteric a. involvement)
3) Neurologic disturbances
4) Skin lesions

24

What serum maker is associated with Polyarteritis Nodosa?

HBsAg (HBV surface antigen)

25

Describe the appearance of early lesions in Polyarteritis Nodosa.

Transmural inflammation

26

Describe the appearance of late lesions in Polyarteritis Nodosa.

Fibrinoid necrosis-->fibrosis

27

How will Polyarteritis Nodosa appear on imaging?

"String of pearls"

- Inflammation leads to weakening of the vessel and "ballooning"
- Fibrosis causes contracture of vessel
- Alternating pattern looks like a pearl necklace

28

How is Polyarteritis Nodosa treated?

1) Corticosteroids
2) Cyclophosphamide

29

What is Kawasaki Disease?

Medium vessel vasculitis that classically effects CHILDREN

30

What arteries are commonly involved in Kawasaki Disease?

Coronary arteries

31

How does Kawasaki Disease present?

Asian child less than 4 y/o with:
- Fever
- Conjunctivitis
- Erythematous rash on palms and soles
- Cervical lymphadenopathy

32

What does coronary a. involvement in Kawasaki Disease lead to?

1) MI
2) Aneurysm

****This is a condition that will cause a MI in a child*****

33

How is Kawasaki Disease treated?

1) ASPIRIN-- seems odd b/c ASA is contraindicated in kids with an apparent viral illness (Reye Syndrome)
2) IVIG

34

What is Buerger Disease?

Medium vessel, necrotizing vasculitis involving the digits

35

What is Buerger Disease associated with?

SMOKING--smoking cessation= remission

36

How does Buerger Disease present?

Ulceration, gangrene, and autoamputation of fingers and toes

37

What is Wegner Granulomatosis?

Small vessel, necrotizing granulomatous vasculitis involving:
- Nasopharynx
- Lungs
- Kidneys

38

What is the classic presentation of Wegner Granulomatosis?

Middle aged male with:
- Sinusitis/ nasopharyngeal ulceration
- hemoptysis
- bilateral nodular infiltrates
- hematuria

39

What serum marker is associated with Wegner Granulomatosis?

c-ANCA

40

How is Wegner Granulomatosis treated?

Cyclophosphamide and steroids

41

What is Microscopic Polyangiitis?

Small vessel, necrotizing vasculitis involving lung and kidney

42

How does Microscopic Polyangiitis differ from Wegner Granulomatosis?

NO nasopharyngeal involvement

43

What serum marker is associated with Microscopic Polyangiitis?

p-ANCA

44

How is Microscopic Polyangiitis treated?

Corticosteroids and cyclophosphamide

45

What is Churg-Strauss Syndrome?

Small vessel, necrotizing granulomatous inflammation involving the heart and lungs

46

What serum marker is associated with Churg-Strauss Syndrome? How does this correlate with disease severity?

p-ANCA

*Direct correlation between p-ANCA levels and severity of disease*

47

How does Churg-Strauss Syndrome differ from Microscopic Polyangiitis?

1) Presence of granulomas
2) Asthma often present
3) Eosinophilia often present

48

What is Henoch-Schonlein Purpura?

Small vessel vasculitis due to IgA immune complex deposotion

49

What is unique about Henoch-Schonlein Purpura?

Most common vasculitis in children

50

How does Henoch-Schonlein Purpura present?

- Palpable purpura on buttocks and legs
- GI pain and bleeding
- Hematuria

****Usually all following a URI*

51

What causes the hematuria seen in Henoch-Schonlein Purpura?

IgA nephropathy

52

How is Henoch-Schonlein Purpura treated?

Typically it is self-limited but severe cases are treated with steroids

53

What is the classic blood pressure that defines HTN?

140/90 mmHg

*Note the either the systolic or diastolic has to be elevated for the diagnosis, BOTH not required

54

What are the two broad categories of HTN?

Primary and secondary

55

What is primary HTN?

HTN of unknown etiology (most common)

56

List the risk factors associated with primary HTN.

Age
Race (African American)
Obesity
Stress
Inactivity
High Na+ diet

57

What is secondary HTN?

HTN due to a known cause

58

What is the most common cause of secondary HTN?

Renal artery stenosis

59

Describe the stenosis of the renal artery increases blood pressure.

1) Stenosis= decreased blood flow to glomerulus
2) Juxtaglomerular apparatus (JGA) responds by secreting renin
3) Renin= Angiotensinogen--> Angiotensin I
4) ACE= Angiotensin I-->Angiotension II

ATII increases blood pressure

60

How does angiotensin II increased blood pressure?

1) Contraction of arteriolar smooth muscle= increased TPR
2) Increases Aldosterone secretion from adrenal cortex

61

How does Aldosterone increase blood pressure?

- Increased Na+ reabsorption in the renal DCT
- Water follows Na+ to increase plasma volume

62

What are the unique features of renal artery stenosis causing HTN?

1) Increases plasma renin w/ HTN (normally, would be low)
2) Unilateral atrophy of the affected kidney

63

What are the two most common causes of renal artery stenosis? What patient populations are these most common in?

1) Atherosclerosis (elderly males most commonly)
2) Fibromusuclar dysplasia (young females)

64

What is Fibromuscular dysplasia?

Developmental defect of the BV wall resulting in an irregular thickening (esp. affecting the renal a.)

65

What is benign HTN?

HTN seen in most individuals
- Clinically silent
- Vessels/ organs damaged slowly over time

66

What is malignant HTN?

Acute and severe elevation in BP that presents with end-organ damage

67

What organs are classically affected by malignant HTN/ what are the signs of malignant HTN?

1) Acute renal failure
2) Headache
3) Papilledema

****This is a medical emergency****

68

What is the definition of arteriosclerosis?

"Hard arteries" caused by thickening of the BV wall

69

What is atherosclerosis?

Specifically refers to intimal plaque formation in medium-sized vessels that obstructs blood flow

70

Describe the composition of an intimal plaque seen in atherosclerosis.

- Necrotic lipid (cholesterol) core
- Fibromusuclar cap
- Undergoes dystrophic calcification

71

What vessels are specifically affected by atherosclerosis?

1) Abdominal aorta
2) Coronary arteries
3) Popliteal arteries
4) Internal carotids

72

What are the modifiable risk factors for atherosclerosis?

1) HTN
2) Hypercholesterolemia
3) Smoking
4) DM

73

What are the non-modifiable risk factors for athersclerosis?

1) Age
2) Gender (males and postmenopausal females)
3) Genetics

74

Describe the pathogenesis of atherosclerosis.

1) Damage to the endothelium allows lipids to leak into the tunica intima
2) Lipids are oxidized and consumed by macrophages-->"foam cells"
3) Inflammation and healing leads to deposition of ECM and proliferation of smooth muscle

75

What are the morphologic stages of atherosclerosis?

1) Fatty streak
2) Atherosclerotic plaque

76

How much stenosis is required before clinically manifestations of atherosclerosis are seen?

70%

77

What are the four broad complications of atherosclerosis?

1) Stenosis of medium-sized vessels/ ischemia of supplied organs
2) Plaque rupture with thrombosis
3) Plaque rupture with embolism
4) Aneurysm

78

What are the common manifestations of medium vessel stenosis seen in atherosclerosis?

- PVD
- Angina
- Ischemic bowel disease

79

What are the two most common outcomes of atherosclerotic plaque rupture and thrombosis?

- MI
- CVA (ischemic)

80

How can you identify an atherosclerotic plaque emboli?

Cholesterol crystals that look like spikes

81

Why does atherosclerosis lead to BV weakening and aneurysm?

- Plaque prevent oxygen diffusion through vessel wall
- Hypoxi/ ischemia= weakness

Aneurysm results

82

What is arteriolosclerosis?

Narrowing of the small ARTERIOLES

83

What are the two types of arteriolosclerosis?

1) Hyaline
2) Hyperplastic

84

What is hyaline arteriolosclerosis?

Proteins leaking into arteriole wall producing vascular thickening

85

How does hyaline arteriolosclerosis appear on microscopy?

Pink

86

What usually causes hyaline arteriolosclerosis?

1) Benign HTN
2) DM

87

What is the typical consequence of hyaline arteriolosclerosis?

- Reduced vessel caliber with end-organ damage
- Typically the KIDNEY
- Progressive to chronic renal failure

88

What is hyperplastic ateriolosclerosis?

Thickening of the arteriole wall by hyperplasia of smooth muscle

89

What typically causes hyperplastic arteriolosclerosis?

Malignant HTN

*Think of this as the vessel thickening to retain all the blood that is being pushed out*

90

What is the typical consequence of hyperplastic arteriosclerosis?

- Reduced vessel caliber with end organ damage
- Causes ACUTE renal failure

91

How is the gross appearance of a failed kidney from hyperplastic arteriolosclerosis described?

"Flea bitten"

92

What is Monckeberg Medial Calcific Sclerosis?

Calcification of the tunica MEDIA in medium sized arteries

93

What is unique about Monckeberg Calcific Sclerosis?

NON-OBSTRUCTIVE i.e. not clinically significant

94

Where are you most likely to encounter Monckeberg Calcific Sclerosis?

Incidental finding on a mammogram

95

What is an aortic dissection?

Intimal tear with disscetion of blood through the tunica media

96

Where does aortic dissection typically occur? Why?

Proximal 10cm of the aorta b/c of thickened diffusion barrier

97

What two things are required for an aortic dissection?

1) High stress
2) Weakness of the tunica media

98

What causes weakness of the tunica media?

1) HTN
2) Inherited connective tissue defects

99

How does HTN lead to weakness of the tunica media?

- HTN--> hyaline arteriolosclerosis
- hyaline arteriolosclerosis effects the VASA VASORUM
- Limited blood flow/ ischemia of vessel wall

100

What are the two inherited connective tissue defects that lead to aortic aneurysm?

1) Marfan's (Fibrillin mutation)
2) Ehler's Danlos Syndrome (Collagen mutation)

101

How does an aortic dissection present?

Sharp, tearing chest pain that radiates to the back

102

What are the major complications of an aortic dissection?

1) Pericardial tamponade
2) Fatal hemorrhage
3) Obstruction of branching arteries

103

How does an aortic dissection cause a pericardial tamponade?

Dissection "back" into the heart causes blood to fill the pericardial sac

104

What is the most common cause of death from aortic dissection?

Pericardial tamponade

105

What is a thoracic aneurysm?

Balloon-like dilation of the thoracic aorta

106

What classically causes throacic aneurysms?

Tertiary syphilis
- Endarteritis of the vasa vasorum
- Luminal narrow

107

How is the gross morphology of the aorta described in a patient with tertiary syphilis?

"Tree bark"

108

What is the major complication of a thoracic aneurysm?

- Proximal aneurysm causing dilation of the aortic valve
- Causes aortic valve insufficiency

109

What are some of the more minor complications of a thoracic aneurysm?

1) Compression of mediastinal structures
2) Thrombosis/ embolism

*Aneurysm causes a disruption of normal laminar flow, which inappropriately activates the coagulation cascade.

110

What is an abdominal aortic aneurysm? Where is this seen?

- Balloon-like dilation of the abdominal aorta
- BELOW the renal arteries but ABOVE the common iliac a.

111

What is the primary cause of an AAA?

Atherosclerosis

*Increases the diffusion barrier leading to weakness and atrophy of the BV wall

112

What is the classic patient with an AAA?

- Male
- Older than 60
- Smoker
- HTN

113

How does an AAA present?

Pulsatile abdominal mass that grows with time

114

What is the major complication of an AAA?

Rupture

115

How does a ruptured AAA present?

Triad of:
1) Hypotension
2) Pulsatile abdominal mass
3) Flank pain

116

What is a hemangioma?

Benign tumor of blood vessels

117

What is the common presentation for a hemangioma?

Seen in kids:
- Present at birth
- Regresses during childhood

118

What areas are most affected by hemangiomas in kids?

Skin and liver

119

What is an angiosarcoma?

Malignant proliferation of endothelial cells

120

What are the common sites affected by angiosarcomas?

Skin
Breast
Liver

121

What three things are liver angiosarcomas associated with?

1) PVC
2) Arsenic
3) Thorostat

122

What is Kaposi Sarcoma?

Low-grade malignant proliferation of endothelial cells associated with HHV-8 viral infection

123

How does Kaposi Sarcoma present?

Purple plaques, patches, and nodules on the skin

*May also involve visceral organs

124

What three patients is Kaposi sarcoma classically seen in?

1) Older Eastern European males
2) AIDS
3) Transplant

125

How do you treat Kaposi Sarcoma in older Eastern European males?

Surgical removal

126

How do you treat Kaposi Sarcoma in AIDS patients?

Anti-retroviral therapy

127

How do you treat Kaposi Sarcoma in transplant patients?

Decrease immunosuppresion