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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
giant cell arteritis granulomas
26
what is pauci immune
non infectious vasculitis where you dont see any deposition of anything in the vessels
27
treatment of PAN
steroids and antiinflammatory, immunosuppressive Treat HBV
28
what does wegners affect
lungs, kidney, upper respiratory tract
29
microscopic polyangiitis note: extravasated RBCs and tons of neutrophils
30
what is vasclutitis
inflammation and necrosis of blood vessels (arteries, veins, capillaries)
31
how do you monitor an autoimmune vasculitis
ANCA MPO and PR3 titers, trends with disease activity
32
localized form of microscopic polyangiitsi
leukocytoclastic vasculitis cutaenous necrotizing vasculitis **palable purpura**
33
clinical features of takayasu
constitutiona high BP, asymmetry, weak pulse occular disturbances, cardaic symptoms, CHF, syncope, dizziness
34
ojo a lymphocytes breaking through the wall PAN
35
what is churg strauss syndrome
systemic vasculitis with prominent eosinophilia that occurs in young persons with asthma or allergy
36
typical takayasu patient
young women under 50 **pulseless disease**
37
classic patient presentation of giant cell arteritis
sudden blindness, temporal pain, headache
38
giant cell arteritis
39
what are causes of small vessel vasculitis continued
CVD, malignancy, mixed cryoglobulinemia radiation GPA, wegners lymphomatoid granulomatosis behcet disease duerger disease vascular allograft rejection
40
epidemiology of giant cell arteritis
older people 70-80 women\>men
41
pathogenesis of giant cell arteritis
a form of T cell mediated proinflammatory cytokintes (TNF, HLA-DR4)
42
what is the most common form of vasculitis
giant cell arteritis
43
pathology of buergers disease
acute inflammation thrombosis, infarcts segmental (acute or chronic) gangrene, amputation
44
treatment of giant cell artritis
steroids, anti TNF
45
left - vasculitis right - necrotizing granuloma GPA
46
what vasculitis is associated with HBV
PAN
47
giant cell arteritis mixed inflammation and necrosis
48
gross appearance of purpura in microscopic polyangiitis
49
what part of the body is immune from PAN
lungs
50
PAN fibrinoid necrosis
51
what are types of small vessel vasculitis
granulomatosis with polyangiitis (GPA) (WG) churg strauss syndrome microscopic polyandiitis
52
pathogenesis of noninfectious vasculitis
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
pathology of takayasu arteritis
aorta and branches thick intima, thick wall panarteritis, with giant cells luminal narrowing, scarring **Pulseless disease**
54
pulseless disease
takayasu
55
what is leukocytoclasis
cell death
56
pathology of churg strauss
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
treatment of takayasu
steroids
58
left - normal right - giant cell arteritis fragmentation of elastic lamina
59
giant cell arteritis
60
what arteries does giant cell arteritis affect
temporal artery, cranial, aorta
61
buergers syndrome
62
PAN kidney
63
pathology of GPA
necrotizing vasculitis. parenchymal/geographic necrosis granulomatous inflammation crescentic glomerulonephritis small arteries, veins and capillaries
64
what is microscopic polyangiitis
group of inflammatory vascular lesions affecting small vessels that are thought to represent a response to exogenous substances
65
takayasu
66
what are types of large vessel vasculitis
giant cell arteritis takayasu arteritis
67
symptoms of kawasaki
affects coronary arteries, aneurysms, rupture, death
68
takayasu left - intimal proliferation thick intima right
69
what is takayaso arteritis
inflammatory diesase of unknown etiology of large arteries (aorta and branches)
70
what is polyarteritis nodosa
acute necrotizing vasculitis of medium sized muscular arteries
71
etiology of kawasaki and treatment
delayed HS t cell reaction infection with parvovirus B19 IVIG and aspirin
72
PAN fibrinoid necrosis, mixed inflammatory cells
73
clinical features of giant cell arteritis
benign, self limiting 6-12 mo muscle aches, **polymyalgia rheumatica** **visual symptoms, diplopia, blindness**

RC Test 4 (10 decks)

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