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RC Test 4 > B4.071 - Vasculitides > Flashcards

B4.071 - Vasculitides Flashcards

1
Q

clinical features of GPA

A

males>F

hematuria, proteinuria, sinusitis, pneumonitis pulmonary infiltrate with cavitation

PR3-ANCA (cytoplasm)

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2
Q

pathology of microscopic polyangiitis

A

arterioles, small arteries

MPO-anca

PR3 - anca

kidney, RPGN, renal failure

skin involvement

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3
Q

pathology of giant cell arteritis

A

cordlike, nodular vessel with narrow lumen. Granulomatous inflammation, plasma cells, MO, eos, Multinucleated giant cells, fibrosis of media and thick intima

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4
Q

microscopic polyangiitis is associated with what

A

drugs (penicillin, thiazides), bacteria, viruses, TB, endocarditis

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5
Q

pathology of microscopic polyangiitis

A

fibrinoid necrosis, acute inflammation, extravasation of RBCs

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6
Q

epidemiology of polyarteritis nodosa &

etiology

A

men>women

white

immune complex mediated

30% of PAN pts have HBV

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7
Q

what is kawasaki disease

A

acute, necrotizing vasculitis of incancy and early childhood, with fever, rash, conjuctival and oral lesions (strawberry toungue) and lymphadenitis

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8
Q

what is PR3-ANCA

A

anti protease 3 (c-ANCA) Seen in granulomatosis iwth polyangiitis, formerly known as Wegeners granulomatosis

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9
Q

generalized form of microscopic polyangiitis

A

systemic HS

asociated with vascular disease, SLE, RA, SS

HSP, malignancy, dysproteinemis

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10
Q

what are types of medium vessel vasculitis

A

polyarteritis nodosa kawasaki disease

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11
Q

etiology of vasculitis

A

Infectious agents HBV, HSV, CMV, Parvovirus serum sickness bacterial antigens autoantibodies: ANCA

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12
Q

clinical features of PAN

A

young adults

acute, subacute or chronic

varies with affected site (kidney, Ht, liver, GI)

constitutional symptoms, infarcts, necrosis

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13
Q
A

giant cell arteritis

narrow lumen, inflammation and giant cells

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14
Q

clinical features of buergers syndrome

A

25-40 yo

men, smokers

intermittend claudication, raynaud syndrome

painful ulcers, gangrene of toes

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15
Q

pathogenesis of beurgers disease

A

smoking

cell mediated immunity, collagen types 2 and 3

genetic factors, HLA A9, B5

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16
Q
A

microscopic polyangiitis

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17
Q

what is thromboangiitis obliterans

A

buerger disease

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

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18
Q

what is wegners granulomatosis aka GPA

A

systemic necrotizing granulomatous vasculitis

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19
Q

who gets buerger disease

A

smokers, young middle aged men

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20
Q

what is MPO-ANCA

A

antimyeloperoxidase (p-ANCA) seen in microscopic polyangiitis and Churg Strauss syndrome

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21
Q

treatment of GPA

A

steroids

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22
Q

what is ANCA

A

autoantibodies against neutrophil granules, monocyte lysosomes, endothelial cells

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23
Q

what is giant cell arteritis

A

focal, chronic, granulomatous inflammation of temporal artery

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24
Q

treatment of churg strauss

A

steroids

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25
Q
A

giant cell arteritis

granulomas

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26
Q

what is pauci immune

A

non infectious vasculitis where you dont see any deposition of anything in the vessels

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27
Q

treatment of PAN

A

steroids and antiinflammatory, immunosuppressive

Treat HBV

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28
Q

what does wegners affect

A

lungs, kidney, upper respiratory tract

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29
Q
A

microscopic polyangiitis

note: extravasated RBCs and tons of neutrophils

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30
Q

what is vasclutitis

A

inflammation and necrosis of blood vessels (arteries, veins, capillaries)

31
Q

how do you monitor an autoimmune vasculitis

A

ANCA MPO and PR3 titers, trends with disease activity

32
Q

localized form of microscopic polyangiitsi

A

leukocytoclastic vasculitis

cutaenous necrotizing vasculitis

palable purpura

33
Q

clinical features of takayasu

A

constitutiona

high BP, asymmetry, weak pulse

occular disturbances, cardaic symptoms, CHF, syncope, dizziness

34
Q
A

ojo a lymphocytes breaking through the wall

PAN

35
Q

what is churg strauss syndrome

A

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

36
Q

typical takayasu patient

A

young women

under 50

pulseless disease

37
Q

classic patient presentation of giant cell arteritis

A

sudden blindness, temporal pain, headache

38
Q
A

giant cell arteritis

39
Q

what are causes of small vessel vasculitis continued

A

CVD, malignancy, mixed cryoglobulinemia radiation GPA, wegners lymphomatoid granulomatosis behcet disease duerger disease vascular allograft rejection

40
Q

epidemiology of giant cell arteritis

A

older people 70-80 women>men

41
Q

pathogenesis of giant cell arteritis

A

a form of T cell mediated proinflammatory cytokintes (TNF, HLA-DR4)

42
Q

what is the most common form of vasculitis

A

giant cell arteritis

43
Q

pathology of buergers disease

A

acute inflammation

thrombosis, infarcts

segmental (acute or chronic)

gangrene, amputation

44
Q

treatment of giant cell artritis

A

steroids, anti TNF

45
Q
A

left - vasculitis

right - necrotizing granuloma

GPA

46
Q

what vasculitis is associated with HBV

47
Q
A

giant cell arteritis

mixed inflammation and necrosis

48
Q
A

gross appearance of purpura in microscopic polyangiitis

49
Q

what part of the body is immune from PAN

50
Q
A

PAN

fibrinoid necrosis

51
Q

what are types of small vessel vasculitis

A

granulomatosis with polyangiitis (GPA) (WG) churg strauss syndrome microscopic polyandiitis

52
Q

pathogenesis of noninfectious vasculitis

A

immune complex deposition in vessel wall antineutrophil cytoplasmic antibodies (ANCA) Direct attack of endothelial cells by circulating endothelial Abs Cell mediated immunity (T cell)

53
Q

pathology of takayasu arteritis

A

aorta and branches

thick intima, thick wall

panarteritis, with giant cells

luminal narrowing, scarring

Pulseless disease

54
Q

pulseless disease

55
Q

what is leukocytoclasis

A

cell death

56
Q

pathology of churg strauss

A

necrotizing vasculitis of small/medium sized vessels

granulomas, eosinophilia

ubiquitous similar to microscopic angiitis or PAN

MPO-ANCA (para nuclear)

lungs, kidneys,m Ht, spleen, liver, CNS

57
Q

treatment of takayasu

58
Q
A

left - normal

right - giant cell arteritis

fragmentation of elastic lamina

59
Q
A

giant cell arteritis

60
Q

what arteries does giant cell arteritis affect

A

temporal artery, cranial, aorta

61
Q
A

buergers syndrome

62
Q
A

PAN

kidney

63
Q

pathology of GPA

A

necrotizing vasculitis. parenchymal/geographic necrosis

granulomatous inflammation

crescentic glomerulonephritis

small arteries, veins and capillaries

64
Q

what is microscopic polyangiitis

A

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

65
Q
66
Q

what are types of large vessel vasculitis

A

giant cell arteritis takayasu arteritis

67
Q

symptoms of kawasaki

A

affects coronary arteries, aneurysms, rupture, death

68
Q
A

takayasu

left - intimal proliferation

thick intima right

69
Q

what is takayaso arteritis

A

inflammatory diesase of unknown etiology of large arteries (aorta and branches)

70
Q

what is polyarteritis nodosa

A

acute necrotizing vasculitis of medium sized muscular arteries

71
Q

etiology of kawasaki and treatment

A

delayed HS t cell reaction

infection with parvovirus B19

IVIG and aspirin

72
Q
A

PAN

fibrinoid necrosis, mixed inflammatory cells

73
Q

clinical features of giant cell arteritis

A

benign, self limiting 6-12 mo

muscle aches, polymyalgia rheumatica

visual symptoms, diplopia, blindness

RC Test 4 (10 decks)

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