Path Lecture: Vessels 3

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1

what is an AAA usually secondary to

atherosclerosis of superior mes arteries, renal arteries coming off with increased turbulence and things that cause medial degeneration/necrosis that lead to bulging and rupture

2

if AAAs are large enough what will they be like

pulsatile

3

what is the borderline width of the AAAs

4-5cm

4

if the AAAs are less than 4 cm what do you do

watch

5

if the AAAs are more than 5cm what is your chance of rupture per year

25%

6

if the AAAs are 4-5cm what is your chance of rupture per year

1% per year

7

what is an aortic dissection

pressurized blood passing through an intimal defect and traveling along the media, forming a blood filled chamber within the vessel wall

8

where are aortic dissections common

aortic arch

9

what will happen in an aortic dissection

blood starts burrowing and splays apart the medial and causes dissection

10

what does a type A AD involve

dissection in ascending

11

what types of DeBakey dissections are type A

DB I and DB II

12

where does a DB I dissection take place

all over

13

where does a DB II dissection take place

ascending

14

where does a DB III take place

thoracic and abdominal (descending)

15

if there is a very proximal dissection where can it get

pericardium and cause tamponade

16

what do people with aortic dissections get

stents or replaced aorta

17

what are the large vessel vasculidities

granulomatous diseases: giant cell arteritis and takayasu's arteris

18

what are the medium vessel vasculidities

immune complex mediated: polyarteritis nodosa

anti-endothelial cell Abs: Kawasaki disease

19

what are the small vessel vasculidities

vasculitis without asthma or granulomas: microscopic polyangiitis

granulomas, no asthma: Wegener's

eosinophilia, asthma, and granulomas: Churg-StrausS syndrome

20

what do most small vessel vasculidities usually involve

paucity of immune complexes often with ANCA

21

what are the immune complex mediated vasculidities

SLE vasculitis (SLE)

HSP (IgA)

cryoglobulin vasculitis (CV)

other (goodpasture's)

22

how do vasculidities normally present

generally: malasie, arthritis, myalgias, vague

23

what are immune mediated vasculidities important to determine nonimmune mediated ones from

B cells react to endothelial cells or infection so that you decided to put them on immunosuppresant drugs

24

what type of HS reactions are most immune mediated vasculitis

noninfectious type 3

25

what is an example of how hep B can cause a vasculitis

hep B surface Ag can incite hep B surface Ab which can deposit in polyarteritis nodosa

26

what will polyarteritis nodosa look like

nodular with multiple inflammation sites

27

where will lupus nephritis immune antibodies deposit

glomeruli

28

what type of immunofluorescent pattern will be seen in lupus nephritis and where

glomeruli, granular pattern

29

what are the immune complex associated vasculidities due to that are NOT due to infection

drug HS: mount a response and it deposits

30

what are the types of special antibodies that can cause arteritis

ANCA (antineutrophil cytoplasmic antibodies)
Anti-endothelial cell antibodies

31

what are the categories of ANCA

antiprotienase3
antimyeloperoxidase

32

what is the antiproteinase3 assd disease

wegener's

33

where is MPO normally

in the lysosome

34

what are the big anti-myeloperoxidase assd disease

microscopic polyangitis
churg strass syndrome

35

what are the anti-endothelial cell antibody assd disease

kawasaki

36

how old are people that get giant cell (temporal) arteritis

over 50

37

where does giant cell arteritis take place

temporal arteries and opthalmic artery

38

what is the vasculitis like in giant cell arteritis

vasculitis with inflammatory cells in intima and media, characteristic finding is giant cells

39

what is disrupted in giant cell arteritis

IEL: stains black

40

what are the clinical features of giant cell artteritis

can range from causing blindness to feeting

malaise, fever, diplopia

41

is giant cell (temporal) arteritis something that you want to wait on

NO

42

what would you treat giant cell arteritis with

steroid

43

what people normall present with takayasu arteritis

under 50 and 80% female

44

where does takayasu arteritis normally affect

aortic arch: 3 major vessels and also hit coronary and renal arteries

45

what is the histology of takayasu arteritis like

marked lumenal steonsis in the areas

46

what do you see in takayasu arteritis in radiology

blood trickling through

47

what type of inflammation will be present in takayasu arteritis

granulomatous (with or without giant cells)

48

what levels of the vessel are expanded in takayasu arteritis

expansion of adventitia

medial HYPERPLASIA

intimal thickening

49

what are the clinical features of takayasu's

reduced upper extremity BP due to occlusion, ocular deficits, lower pulses

if coronary: MI

if kidneys: HTN

50

what is pulseless disaes

takayasu disease

51

what age do people get PAN

young adults

52

what types of arteries get PAN

medium

53

what are some arteries that get PAN

renal vessels
mesentaric vessels

54

does PAN affect the pulmonary vessels

typically not

55

what is the histology of PAN

segmental fibrinoid necrosis

NODULAR

56

what are the lcinical features of PAN

malaise and particular site like mesenteric could be infarcted

57

what infection could cuase PAN

hep B

58

what is another name for kawasakis

mucocutaneous lymph node syndrome

59

how old are people who have kawasaki

under 4

60

where does kawasaki's affect

"kiddy coronary": can get some of the larger vessels

61

what does the histology of kawasaki's look like

fibrinoid necrosis of vessels

62

where is the antibody directed against in kawasaki's

endothlium

63

what cells are activated in kawasaki's

B cells!

64

if kawasaki is in coronaries, what do you have a risk of

aneurym/dilation and risk of rupture

65

what are the clinical features of kawasaki's

vague but you can hvae cutaneous expressions like conjuntiviities, oral erythema, edema of heands and feet, cervical LN enlargment

66

what are the anca assd vasculitises

microscopic polyangitis
wegener grnaulomatosis
churg strauss

67

what is another name for microscopic polyangitis

leukocytoclasctic vasculitis

68

what type of ANCA is assd with microscopic polyangitis

MPO

69

where is the site of microscopic polyangiitis

capillaries, arterioles, venules

70

what are the clinical features of microscopic polyangitis

necrotizing glomerulonephritis and pulmonary capillaritis

present with proteinuria and hematuria, possibly hemoptysis

71

what is another name for wegener granulomatosis

anca-assd grnaulomatosis

72

what is the prototype age of wegener's

40+ years

73

what is the site of wegener's

small vessels (upper respiratory tract, lungs, and glomeruli

74

what is the histology of wegeners granulomatosis

necrotizing granulomatous vasculitis with epithelioid histiocytes, crescentic glomerulonephritis in kidney forming crescents under bowmna's capsule

75

what are the clinical presentations of wegener's granulomatosis related to the lungs

problems with pneumonitis (parts of alveoli) as well as granulomatous inflammation of upper airway (chronic sinusitis) that can affect nasopharynx or all the way to the tympanic membrane

76

what other than lungs can wegener's affect

glomerulonephrtis

77

what ANCA is assd with wegener's

protein 3

78

what are the treatments for wegeners

steroids or anything tht knocks down the inflammatory process

79

what is the Churg Strauss syndrome anca

MPO

80

what is another name for churg strauss syndrome

allergic granulomatosis and angiitis OR eosinophilic granulomatosis with polyangitis

81

what is the site of churg strauss

small vessels and lungs

82

what will the histology of churg strauss look ikke

perivascular eosinophilia, not neutrophils!

83

what are the clinical features of churg strauss

rhinitis, lung problems, CMs

84

what is another name for thromboangiitis obliterans

buerger's disease

85

what people get thromboangiits obliterans

smokers under 35

86

what is the histology of thromboangiits obliterans like

transmural chronically acute inflammation with lumenal stenosis

87

what arteries are affected by thromboangitis obliterans

tibial and radial arteis

88

what are the clincal features of thromboangiits obliterans like

complicated clotting vasculitis, acute thrombosing vasculitis

causes GANGRENOUS NECROSIS OF EXTREMITIES

89

why do most thromboangiitis obliterans people smoke

because the smoke causes HS reaction to tobacco that irritates the vessels

90

what are the ANCAs

anti-proteinase 3
anti-MPO
anti-endothelial

91

what is another name for c-ANCA

anti proteinase 3

92

what is another name for p-ANCA

anti-MPO

93

what disease is anti proteinase 3 assd with

wegeners

94

what is anti MPO associated with

microscopic polyangitis and churg strauss

95

what is anti-endothelial ANCA assd with

kawasaki

96

what are the large vessel vasculitdies

giant cell temporal arteritis
takayasu arteritis

97

what type of inflammation will giant cell arteritis have

granulomatous

98

what will the symptoms of giant cell temporal arterits have

headach and blindness

99

what will the symptoms of takayasu arteritis be

PULSELESS DISEASE

100

what type of inflammation will show in takayasu

mononuclear

101

what are the medium vessel vasculidities

polyartertis nodosa
kawasaki diesase

102

what type of necrosis is in the MEDIUM VESSEL VASCULIDITIES

fibroniod necrosis

103

what can PAN be from

hep B infection

104

what is another name for kawasakis

mucocutaneous LN syndrome

105

who gets kawasaki and where

kids, coronary artery aneurysms

106

what are the small vessel vasculidities

microscopic polyangitis
wegener granulomatosis
churg strauss
buerger

107

what type of necrosis will be in microscopic polyangitis

fibrinoid necrosis of small vessels and capillaries, leukocytoclasic vasculitis

108

what type of inflammation will be assod with wegeners

grannulomatous

109

what type of inflammation will be assd with CS

allergic eosinophilic granulomatosis and angiitis

110

what is the ANCA of microscopic polyangitis

mpo

111

what is the anca for wegeners

pr3

112

what is the anca for CS

mpo

113

where does microscopic polyangitis affect

necrotizing glomerulonephritis and pulmonary capillaries

114

where does wegener's affect

uRT, LRT
glomerulonephritis

115

what does churg-strauss affect

astham, rhinitis

116

what types of vessls does buerger's disease affect

medium and small vessels

117

what makes buerger's worse

SMOKING

118

what are the angioinvasive vasculitises

invasive fungal: aspergillus and mucor

119

what do invasive fungal sinusitises lead to

direct invasion: weakening/damage of vascular walls, thrombus, and infection