Vasculitides Flashcards

1
Q

Gaint cell (temporal) arteritis: who and what age?

A

Female >50

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2
Q

Gaint cell (temporal) arteritis: symptoms? (3)

A

unilateral headache
jaw claudication
possible temporal artery tenderness

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3
Q

Gaint cell (temporal) arteritis: important complication?

A

irreversible blindness die to AION (anterior ischemic optic neuropathy)

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4
Q

irriversible blindness in Gaint cell (temporal) arteritis: cause?

A

anterior ischemic optic neuropathy (AION)

= interruption of blood flow to the front (anterior) of the optic nerve

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5
Q

Gaint cell (temporal) arteritis: associated with which other disease?

A

polymyalgia rheumatica

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6
Q

which arteritis is associated with PMG?

A

Gaint cell (temporal) arteritis

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7
Q

which arteritis is most common in female >50?

A

Gaint cell (temporal) arteritis

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8
Q

unilateral headache, tenderness of temporal artery, jaw claudication

what can it be?

A

Gaint cell (temporal) arteritis

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9
Q

which arteritis may lead to irreversible blindness due to AION?

A

Gaint cell (temporal) arteritis

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10
Q

Gaint cell (temporal) arteritis: which artery branches most commonly affected?

A

carotid branches

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11
Q

Gaint cell (temporal) arteritis: what kind of inflammation?

A

focal granulomatous

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12
Q

Gaint cell (temporal) arteritis: which inflammation marker very high?

A

ESR

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13
Q

Gaint cell (temporal) arteritis: levels of which cytokine correlate with disease activity?

A

IL-6

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14
Q

Gaint cell (temporal) arteritis: treatment?

A

high dose glucocorticocoids

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15
Q

Gaint cell (temporal) arteritis: when to apply treatment, before or after biopsy?

A

before

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16
Q

Gaint cell (temporal) arteritis: diagnosis?

A

temporal artery biopsy

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17
Q

Gaint cell (temporal) arteritis: why start treatment before biopsy?

A

to prevent blindness

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18
Q

Takayasu arteritis: in who and what age?

A

asian female <40

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19
Q

Takayasu arteritis: symptoms?

A

weak upper extremity pulses

fever, night sweats, arthritis, myalgias

skin nodules and ocular disturbances

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20
Q

other name for Takayasu arteritis?

A

pulselessness disease

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21
Q

pulselessness disease: what is this?

A

takayasu

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22
Q

takayasu arteritis: pathophysiology?

A

granulomatous thickening/narrowing of aortic arch and proximal great vessels

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23
Q

35 y/o chinese woman
weak upper extremity pulses,
night sweats, myalgias, skin nodules
elevated ESR

suggestive of?

A

takayasu

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24
Q

takayasu: which inflammation marker elevated?

A

ESR

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25
Q

takayasu: treatment?

A

glucocorticoids

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26
Q

granulomatous thickening/narrowing of aortic arch and proximal great vessels

which disease?

A

takayasu

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27
Q

what are the large vessel vasculitides?

A

giant cell arteritis
takayasu

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28
Q

what are the medium vessel vasculitides?

A

Buerger, Kawasaki, polyarteritis nodosa

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29
Q

Buerger: in who?

A

males <40
heavy tabacco smoking

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30
Q

Buerger: other name?

A

thromboangiitis obliterans

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31
Q

Buerger: symptoms?

A

intermittent claudication

may lead to gangrene, autoamputation of digits, superficial nodular phlebitis

Raynaud phenomen often present

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32
Q

man 35, heavy tobacco smoking history

intermittent claudication, gangrene toe, Raynaud phenomen

suggestive of?

A

Buerger

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33
Q

Buerger: pathophysiology?

A

segmental thrombosing vasculitis
vein and nerve involvement

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34
Q

Buerger: treatment?

A

smoking cessation

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35
Q

segmental thrombosing vasculitis
vein and nerve involvement

what is this?

A

Buerger

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36
Q

Kawasaki: in who?

A

Asian kids <4

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37
Q

vasculitis common in asian kids <4?

A

kawasaki

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38
Q

kawasaki: symptoms?

A

CRASH and burn

Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hand and foot changes

and burn: fever

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39
Q

3 y/o japanese kid

fever, cervical adenopathy, desquamating rash, red and swollen feet and hands

suggestive of which vasculitis?

A

kawasaki

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40
Q

rash in kawasaki?

A

polymorphous, desquamating (shedding/scraping off)

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41
Q

what kind of adenopathy in kawasaki?

A

cervical

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42
Q

hand and foot changes in kawasaki?

A

swollen and erythema

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43
Q

conjunctivitis in kawasaki?

A

bilateral non-exudative (no fluids) bulbar

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44
Q

is the conjunctivitis in kawasaki unilateral or bilateral?

A

bilateral

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45
Q

what was kawasaki formerly called?

A

mucocutaneous lymph node syndrome

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46
Q

kawasaki: complications?

A

coronary artery aneurysms; thrombosis or rupture can cause death

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47
Q

what can cause death in kawasaki?

A

coronary artery aneurysms

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48
Q

kawasaki: treatment?

A

IVIG and aspirin

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49
Q

polyarteritis nodosa: in who?

A

middle-aged males

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50
Q

polyarteritis nodosa: seropositive for what in 30% of the cases?

A

Hepatitis B

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51
Q

in which arteritis 30% of patients seropositive for Hep B?

A

polyarteritis nodosa

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52
Q

polyarteritis nodosa: involvement of which systems?

A

cardiovascular, GI, neuro, skin, renal

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53
Q

polyarteritis nodosa: symptoms?

A

GI: abdominal pain, melena
Hypertension, neurologic dysfunction, skin sores, renal damage

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54
Q

52 y/o man with hypertension, abdominal pain, melena, skin sores, proteinuria and hematuria

Hep B seropositivity

suggestive of?

A

polyarteritis nodosa

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55
Q

what system is NOT involved in polyarteritis nodosa?

A

pulmonary

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56
Q

which arteries are typically involved in polyarteritis nodosa?

A

renal and visceral

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57
Q

polyarteritis nodosa: renal angiogram/ateriogram findings?

A

innumerable renal microaneurysms, and spams

‘string of pearl’ appearance

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58
Q

‘string of pearl’ appearance on renal angiogram

which disease?

A

polyarteritis nodosa

59
Q

polyarteritis nodosa: treatment?

A

glucocorticoids
cyclophosphamide

60
Q

polyarteritis nodosa: what is it?

A

small and medium-sized arteries swollen and damaged

serious vasculitis

61
Q

Behcet syndrome: in who?

A

people with Turkish and eastern mediterranean descendent

62
Q

Behcet syndrome: symptoms?

A

aphtous ulcers, genital ulceration, uveitis, erythema nodosum

63
Q

what is erythema nodosum?

A

tender, red bumps, usually found symmetrically on the shins

64
Q

what kind of small vessel vasculitis can cause uveitis?

A

behcet

65
Q

what can behcet be precipitated by?

A

HSV (herpes simplex virus) or parvovirus

66
Q

HSV and parvovirus can trigger which vasculitis?

A

behcet

67
Q

how long do behcet flares last?

A

1-4 weeks

68
Q

which vasculitis comes in flares?

A

behcet
1-4 weeks

69
Q

turkish man with genital ulcers, erythema nodosum and uveitis

suggestive of?

A

behcet

70
Q

what kind vasculitis is behcet?

A

immune complex

71
Q

with which HLA type is behcet associated?

A

HLA-B51

72
Q

which vasculitis is associated with HLA-B51?

A

behcet

73
Q

cutaneous small vessels vasculitis: when does it occur?

A

7-10 days after medication or infection

74
Q

cutaneous small vessels vasculitis: which medication can cause it?

A

penicillins
cephalosporins
sulfonamides

phenytoin

allopurinol

75
Q

penicillins
cephalosporins
sulfonamides
phenytoin
allopurinol

can cause which vasculitis?

A

cutaneous small vessel vasculitis

76
Q

cutaneous small vessel vasculitis: which infections can cause this?

A

HCV (hep C), HIV

77
Q

cutaneous small vessel vasculitis: symptoms?

A

palpable purpura

(7-10 days after certain infection or medication)

78
Q

cutaneous small vessel vasculitis: visceral involvement?

A

no

79
Q

which small vessel vasculitis has no visceral involvement?

A

cutaneous small vessel vasculitis

80
Q

person with palpable purpura, no visceral symptoms
8 days after taking antibiotics

which vasculitis?

A

cutaneous small vessel vasculitis

81
Q

person with palpable purpura
9 days after HCV infection

which vasculitis?

A

cutaneous small vessel vasculitis`

82
Q

what kind of vasculitis is cutaneous small vessel vasculitis?

A

immune complex mediated leukocytoclastic

83
Q

leukocytoclastic vasculitis: other name?

A

cutaneous small vessel vasculitis

84
Q

cutaneous small vessel vasculitis: other name?

A

leukocytoclastic vasculitis

85
Q

what is leukocytoclasis?

A

infiltrating neutrophils causing vascular damage

cutaneous small vessel vasculitis

86
Q

churg strauss: other name?

A

eosinophilic granulomatosis with polyangiitis

87
Q

eosinophilic granulomatosis with polyangiitis: other name?

A

churg-strauss

88
Q

eosinophilic granulomatosis with polyangiitis: symptoms?

A

asthma
sinusitis
skin nodules or purpura
peripheral neuropathy (wrist/foot drop)

89
Q

eosinophilic granulomatosis with polyangiitis: which other organs can be involved?

A

heart, BI, kidney

90
Q

how do call eosinophilic granulomatosis with polyangiitis with renal involvement?

A

pauci-immune glomerulonephritis

91
Q

eosinophilic granulomatosis with polyangiitis: pathophysiology?

A

granulomatous, necrotizing vasculitis with eosinophilic involvement

92
Q

eosinophilic granulomatosis with polyangiitis: antibodies?

A

P-ANCA/MPO-ANCA
IgE increased

93
Q

eosinophilic granulomatosis with polyangiitis: which ANCA?

A

P-ANCA

94
Q

Churg-strauss: which cells particularly involved?

A

eosinophilesc

95
Q

churg-strauss: symptoms?

A

asthma
sinusitis
skin nodules or purpura
foot/wrist drop (neuropathy)

96
Q

person with sinusitis, wrist drop, and skin purpura
P-ANCA positive, elevated IgE

suggestive of?

A

churg strauss (eosinophilic granulomatosis with polyangiitis)

97
Q

which small vessel vasculitis has increased IgE?

A

eosinophilic granulomatosis with polyangiitis

98
Q

granulomatosis with polyangiitis: which 3 systems are involved?

A

upper respiratory tract
lower respiratory tract
renal

99
Q

granulomatosis with polyangiitis: symptoms upper respiratory tract?

A

perforation nasal septum
chronic sinusitis
otitis media
mastoiditis

100
Q

granulomatosis with polyangiitis: symptoms lower respiratory tract?

A

hemoptysis
cough
dyspnea

101
Q

granulomatosis with polyangiitis: renal symptoms?

A

hematuria, red cell clasts

102
Q

person with chronic sinusitis, otitis media, couching, hemoptysis and hematuria

C-ANCA is positive

suggestive of?

A

granulomatosis with polyangiitis

103
Q

involvement of lower/upper respiratory tract and kidneys

C-ANCA positive

suggestive of which vasculitis?

A

granulomatosis with polyangiitis

104
Q

pathological triad of granulomatosis with polyangiitis?

A

focal necrotizing vasculitis
necrotizing granulomas in lung/upper airway
necrotizing glomerulonephritis

105
Q

granulomatosis with polyangiitis: which ANCA?

A

C-ANCA/PR3-ANCA

106
Q

granulomatosis with polyangiitis: CXR?

A

large nodular densities

107
Q

in which vascilitis large nodular densities on CXR?

A

granulomatosis with polyangiitis

108
Q

granulomatosis with polyangiitis: treatment?

A

glucocorticosteroids in combination with rituximab or cyclophosphamide

109
Q

glucocorticosteroids in combination with rituximab or cyclophosphamide

treatment for what?

A

granulomatosis with polyangiitis

110
Q

what does rituximab do?

A

kills b-cells

111
Q

Immunoglobulin A vasculitis: other name?

A

henoch schonlein purpura

112
Q

henoch schonlein purpura: other name?

A

immunoglobulin A vasculitis

113
Q

Immunoglobulin A vasculitis: often follows what?

A

URI (upper resp infection)

114
Q

most common childhood systemic vasculitis?

A

Immunoglobulin A vasculitis
henoch schonlein purpura (HSP)

115
Q

Immunoglobulin A vasculitis: classic triad?

A

HSP

Hinge pain (arthralgia)
Stomach pain
Palpable purpura

116
Q

Immunoglobulin A vasculitis: stomach pain associated with what?

A

intussusception

(invaginatie)

117
Q

Immunoglobulin A vasculitis: where are the palpable purpura?

A

buttocks and legs

118
Q

child with arthalgia, purpura on buttocks and legs, stomach pain

suggestive of?

A

immunoglobulin A vasculitis

119
Q

henoch schonlein purpura: which immunoglobins are involved?

A

IgA immune complexes

120
Q

Hinge pain (arthralgia)
Stomach pain
Palpable purpura

what is this?

A

immunoglobin A vasculitis
or henoch schonlein purpura

121
Q

immunoglobin A vasculitis: treatment?

A

supportive care
possibly glucocorticoids

122
Q

henoch schonlein purpura: treatment?

A

supportive care
possibly glucocorticoids

123
Q

microscopic polyangiitis: presentation?

A

lung: couch, hemoptysis, dyspnea
kidney: hematuria, red clasts
skin: palpable purpura

similar to granulomatosis with poylangiitis without nasopharyngeal involvement

124
Q

microscopic polyangiitis: which 3 systems are involved?

A

lung
kidney
skin

125
Q

microscopic polyangiitis: skin symptoms?

A

palpable purpura

126
Q

microscopic polyangiitis: lung symptoms?

A

coughing, hemoptysis, dyspnea

127
Q

microscopic polyangiitis: renal symptoms?

A

hematuria and red cell clasts

128
Q

how do call kidney involvement in microscopic polyangiitis?

A

pauci-immune glomerulonephritis

129
Q

microscopic polyangiitis: granulomas?

A

no

130
Q

microscopic polyangitis: which ANCA?

A

P-ANCA (MPO-ANCA)

131
Q

what is P-ANCA?

A

anti-myeloperoxidase
(also anti-MPO)

132
Q

microscopic polyangiitis: treatment?

A

cyclophosphamide, glucocorticoids

133
Q

which vasculitides have ANCA?

A
  • eosinophilic granulomatosis with polyangiitis
  • granulomatosis with polyangiitis
  • microscopic polyangiitis
134
Q

which vasculitis have P-ANCA

A
  • eosinophilic granulomatosis with polyangiitis
  • microscopic polyangiitis
135
Q

which vasculitide has C-ANCA?

A

granulomatosis with polyangiitis

136
Q

which vasculitide is similar to granulomatosis with polyangiitis, but without nasopharyngeal involvement?

A

microscopic polyangiitis

137
Q

Mixed cryoglobulinemia: often due to what?

A

infections
especially HCV (Hep C)

138
Q

Mixed cryoglobulinemia: triad?

A

palpable purpura, weakness, arthralgias

139
Q

Mixed cryoglobulinemia: other involvement (outside triad)?

A

peripheral neuropathy, renal disease (glomerulonephritis)

140
Q

what are cryoglobulins?

A

immunoglobulins that precipitate in the cold

141
Q

Mixed cryoglobulinemia: what kind of vasculitis?

A

immune complex
mix of IgG and IgM

142
Q

Mixed cryoglobulinemia: mix of which immunoglobulins?

A

IgG and IgM

143
Q

mix of IgG and IgM immuno complex deposition

which vasculitis?

A

Mixed cryoglobulinemia

144
Q

palpable purpura, weakness, arthralgias

HCV seropositive

suggestive of?

A

Mixed cryoglobulinemia