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RC Test 4 > B4.071 Vasculitides > Flashcards

B4.071 Vasculitides Flashcards

1
Q

what is vasculitis

A

inflammation and necrosis of blood vessels

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2
Q

etiology of vasculitis

A 
infectious agents, mechanical, radiation, toxins
HBV, HSV, CMV, parovirus
serum sickness
bacterial antigen
autoantibodies: ANCA
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3
Q

pathogenesis of “noninfectious” vasculitis

A
  1. immune complex deposition in vessel wall
  2. antineutrophil cytoplasmic antibodies (ANCA)
  3. direct attack of endothelial cells by circulating anti-endothelial Abs
  4. cell mediated immunity (autoreactive T cells)
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4
Q

what are ANCAs antibodies against?

A
  1. neutrophil granules/monocyte lysosomes/endothelial cells
  2. anti-myeloperoxidase (MPO-ANCA) (p-ANCA)
  3. anti-proteinase-3 (PR3-ANCA) (c-ANCA)
  4. other
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5
Q

what diseases are MPO/p-ANCAs associated with

A

microscopic polyangiitis

Churg-Strauss Syndrome

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6
Q

what diseases are PR3/c-ANCAs associated with

A

granulomatosis with polyangiitis (GPA) (wegeners)

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7
Q

why are ANCAs measured?

A

track disease activity

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8
Q

what are some ways that vasculitis is classified

A 
sixe of blood vessel
location
type
nature of inflammatory infiltrate
specific etiological factor
immunopathogenic mechanism
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9
Q

large vessel vasculitides

A

Giant-cell arteritis

Takayasu arteritis

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10
Q

medium vessel vasculitides

A

polyarteritis nodosa

kawaski disease

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11
Q

small vessel vasculitides

A

granulomatosis with polyangiitis (GPA) (wegeners)
churg-strauss syndrome
microscopic polyangiitis

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12
Q

giant cell arteritis

A

focal, chronic, granulomatous inflammation of temporal artery

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13
Q

epidemiology of giant cell arteritis

A

old age (70-80 yr)
women > men
most common form of vasculitis

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14
Q

which arteries does giant cell arteritis primarily affect

A

temporal, cranial, aorta

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15
Q

pathogenesis of giant cell arteritis

A

T cell mediated
proinflammatory cytokines (TNFa)
HLA-DR4

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16
Q

pathology of giant cell arteritis

A

cordlike, nodular vessel w narrow lumen
granulomatous inflammation, plasma cells, multinucleated giant cells
necrosis, fragmentation of elastic lamina
fibrosis of media and thick intima

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17
Q

clinical features of giant cell arteritis

A

benign, self limiting
muscle aches
visual symptoms, diplopia, blindness

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18
Q

treatment of giant cell arteritis

A

steroids

anti-TNF

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19
Q

how does fragmentation of elastic lamina appear on histology?

A

blue stained lamina abruptly stops and occurs in segments rather than being continuous

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20
Q

takayasu arteritis

A

inflammatory disease of unknown etiology in large arteries (aorta and branches)

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21
Q

epidemiology of takayasu

A

young women

autoimmune?

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22
Q

pathology of takayasu

A

aorta and branches involved
thick intima, thick wall
panarteritis with giant cells
luminal narrowing, scarring

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23
Q

why is takayasu called pulseless disease

A

blood flow can’t reach extremities well

pulses cant be found

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24
Q

clinical features of takayasu

A

constitutional symptoms
high BP, asymmetry, weak pulse
ocular disturbances

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25
Q

treatment of takayasu

A

steroid

surgical reconstruction

26
Q

polyarteritis nodosa (PAN)

A

acute necrotizing vasculitis of medium sized muscular arteries

27
Q

epidemiology of polyarteritis nodosa

A

men > women

whites > blacks

28
Q

etiology of polyarteritis nodosa

A

immune complex mediated damage of vessels

30% of PAN patients have chronic hep B

29
Q

pathology of PAN

A

small to medium sized and large vessels
fibrinoid necrosis, mixed inflammatory cells
thrombosis, infarction of organ

30
Q

clinical features of PAN

A

young adults
acute, subacute, or chronis
varies w affected site (kidney, heart, liver, GI)
constitutional symptoms, infarcts, necrosis

31
Q

treatment of PAN

A

steroids
anti inflamm drugs
immunosuppressive agents
control hep B

32
Q

unique characteristic of PAN

A

does NOT affect lungs

all other do

33
Q

Kawasaki disease

A

acute necrotizing vasculitis of infancy and early childhood with fever, rash, conjunctival and oral lesions, and lymphadenitis

34
Q

etiology of kawasaki

A

delayed hypersensitivity T cell reaction

infection with parovirus B19

35
Q

Kawasaki epidemiology

A

85% in kids <5

95% in kids <10

36
Q

clinical features of kawasaki

A

strawberry red tongue

red rashes on hands and feet

37
Q

treatment of kawasaki

A

self limited
IVIg
aspirin

38
Q

more serious complications of kawasaki

A

cardiac symptoms:

-coronary arteries affects, aneurysms, rupture, death

39
Q

granulomatosis with polyangiitis (GPA) (wegeners)

A

systemic necrotizing granulomatous vasculitis of unknown etiology

40
Q

organ systems affected by GPA

A

lungs
kidneys
upper resp tract

41
Q

pathology of GPA

A 
necrotizing vasculitis
parenchymal/geographic necrosis
granulomatous inflammation (T cell)
small arteries, veins, capillaries
crescentic glomerulonephritis (but could potentially not manifest in kidneys)
42
Q

clinical features of GPA

A 
males > females
hematuria, proteinuria (renal disease)
sinusitis (URT)
pneumonitis, pulm infiltrate with cavitation (LRT)
PR3-ANCA (over 90% of cases)
43
Q

treatment of GPA

A

steroids
cyclophosphamide
anti-TNF

44
Q

Churg-Strauss Syndrome

A

systemic vasculitis w prominent eosinophilia that occurs in young persons w asthma or allergy

45
Q

pathology of churg strauss

A

necrotizing vasculitis of small and medium vessels
granulomas, eosinophilia, fibrinoid necrosis
similar to microscropic angiitis or PAN
MPO-ANCA (< half of cases)

46
Q

organ systems affected by churg strauss

A 
lungs
kidney
heart
liver
spleen
CNS
47
Q

differential for churg strauss

A 
PAN
parasitic, fungal infections
GPA
eosinophilic pneumonia syndrome
drug vasculitis
48
Q

treatment for churg strauss

A

steroids

anti-TNF

49
Q

microscopic polyangiitis

A

a group of inflammatory vascular lesions affecting small vessels that are thought to represent a response to exogenous substances
localized and generalized forms

50
Q

localized form of microscopic polyangiitis

A

skin
leukocytoclastic vasculitis
cutaneous vasculitis/venulitis
palpable purpura

51
Q

what is the localized form associated with

A 
drugs
bacteria
viruses
TB
bacterial endocarditis
52
Q

pathology of localized form of microscopic polyangiitis

A

fibrinoid necrosis
acute inflammation (neutrophils)
extravasation of RBCs

53
Q

generalized form of microscopic polyangiitis

A

systemic hypersensitivity polyarteritis

microscopic polyarteritis

54
Q

what is the generalized form associated with

A 
collagen vascular disease
SLE
RA
HSP
malignancy
dysproteinemia
55
Q

pathology of generalized form of microscopic polyangiitis

A

arterioles and small arteries
MPA-ANCA, PR3-ANCA
kidney, rapidly progressing glomerulonephritis and renal failure
skin involvement

56
Q

microscopic features of leukocytoclastic vasculitis (localized microscopic polyangiitis)

A 
fibrinoid necrosis of vessel wall
intramural neutrophilic infiltrates
leukocytoclasis (cell death)
RBC extravasation
involvement of small venules
57
Q

thromboangiitis obliterans (bueger)

A

occlusive inflammatory disease of medium and small arteries in the distal arms and legs

58
Q

epidemiology of buerger

A

smokers, young middle aged men

59
Q

pathogenesis of buerger

A

smoking (tobacco products)
cell mediated immunity, collagen types 2, 3
genetic factors (Israeli, Indians, Japanese)
HLA A9, B5

60
Q

pathology of buerger

A

acute inflamm of medium and small arteries
thrombosis, infarcts
segmental (acute or chronic)
gangrene, amputations

61
Q

clinical features of buerger

A

25-40 y/o men, smokers
intermittent claudication, Raynaud phenomenon (loss of feeling and blueness of extremities)
painful ulcers, gangrene of toes/fingers

RC Test 4 (22 decks)

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