Vasculitis Flashcards

1
Q

what is vasculitis

A

inflammation of blood vessels,

often with ischaemia, necrosis and organ inflammation

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2
Q

what blood vessels can vasculitis affect

A

all of them

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3
Q

what level should eosinophils not exceed?

A

1

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4
Q

which size of vessel has the worst prognosis when affected by vasculitis

A

small vessels

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5
Q

what is primary vasculitis

A

vasculitis caused by an inflammatory response that targets the blood vessels with no known cause

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6
Q

what is secondary vasculitis

A

vasculitis triggered by an infection, drug, toxin or cancer

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7
Q

what do activated T cells do to the blood vessels?

A

promote inflammation, granuloma formation, macrophage activation and differentiation

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8
Q

what activates the T cells

A

inflammatory cytokines released from dendritics

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9
Q

name the 2 forms of large vessel vasculitis

A

takayasu arteritis

giant cell arteritis

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10
Q

name 2 forms of medium vessel vasculitis

A

polyareritis nodosa

kawasaki disease

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11
Q

what 2 categories is small vessel vasculitis divided into?

A

immune complex

ANCA associated

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12
Q

which small vessel vasculitis category is polyangiitis associated with?

A

ANCA-associated

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13
Q

ANCA positive small vessel vasculitis with granulomas, asthma and eosinophilia?

A

egpa

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14
Q

ANCA positive small vessel vasculitis with granulomas only?

A

gpa

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15
Q

ANCA negative small vessel vasculitis with IgA dominant immune deposit

A

henoch schonlein purpura

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16
Q

ANCA negative small vessel vasculitis with no IgA and no serum ceyoglobulin?

A

other ANCA negative vasculitis eg IBD vasculitis

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17
Q

ANCA negative small vessel vasculitis with no IgA immune deposit and has serum cryoglobulin present

A

cryoglobulinaemia

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18
Q

what systemic symptoms are common to all vasculitides

A

fever
malaise
weight loss
fatigue

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19
Q

who tends to get takayasu arteritis

A

asian women under 40

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20
Q

who tends to get giant cell arteritis

A

elderly people

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21
Q

presenting features of giant cell arteritis?

A

carotid bruits
claudication
HT
BP difference in extremities

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22
Q

common symptoms of temporal arteritis

A

polymyalgia rheumatica
unilateral temporal headache
scalp tenderness
jaw claudication

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23
Q

investigations for temporal arteritis

A
ESR
plasma viscosity
CRP
temporal artery biopsy
PET CT
MR angiogram
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24
Q

treatment for temporal arteritis

A

40-60mg prednisolone

consider methotrexate or azathioprine

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25
Q

who gets kawasaki disease?

A

children under 5

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26
Q

most common arteries affected by kawasaki disease? why is this relevant?

A

coronary; can develop aneurysm

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27
Q

where does polyarteritis nodosa typically affect?

A

arteries at vessel bifurcations

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28
Q

which vasculitis is associated with hep b

A

medium vessel

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29
Q

which syndromes should you consider when the respiratory tract is affected?

A

GPA

eGPA

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30
Q

who tends to get gpa?

A

people of northern european descent in their middle age

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31
Q

another name for wegeners?

A

granulomatosis with polyangiitis

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32
Q

another name for churg strauss syndrome

A

eosinophilic granulomatosis with polyangiitis

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33
Q

what would a chest x ray show for gpa?

A

nodules
fixed infiltrates
cavities

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34
Q

ENT features of gpa

A

sinusitis
nasal crusting
mouth ulcers
saddle nose

35
Q

main difference between churg strauss and wegeners

A

get late onset asthma and high eosinophil count in churg strauss

36
Q

which condition is cANCA raised in?

A

granulomatous polyangiitis/ wegeners

37
Q

which condition is pANCA raised in?

A

eosinophilic granulomatous polyangiitis

38
Q

is anti-PR3 associated with gpa or egpa

A

GPA

39
Q

management of ANCA +ve vasculitis

A

early- methotrexate + steroids

systemic - cyclophosphamide +steroids OR 2nd line rituximab + steroids

40
Q

differentials other than vasculitis

A

chronic infection

cancer

41
Q

areas that GCA can affect?

A

temporal artery
aorta
subclavian artery

42
Q

areas that takayasu arteritis affects?

A

aorta

43
Q

why can someone with GCA get claudication symptoms?

A

if subclavian/aorta are involved and they are doing repetitive activity

44
Q

what kind of inflammation exists in large vessel vascultis?

A

granulomatous

45
Q

presenting features of large vessel vasculitis?

A
bruit
blood pressure difference of extremities
claudication
carotodynia
hypertension
46
Q

what percentage of people with GCA have PMR?

A

50%

47
Q

what percentage of people with PMR have GCA?

A

15%

48
Q

symptoms of GCA?

A

unilateral temporal headache
scalp tenerness
jaw claudication

49
Q

when would you do an MR angiogram or PET CT of someone with GCA?

A

if vessels other than the temporal artery are involved

50
Q

what would an MR angiogram show?

A

vessel wall thickening/stenosis/aneurysm

51
Q

how does a PET CT help in someone with GCA?

A

shows where metabolically active cells eg inflamed cells are

52
Q

should you start steroids for GCA before or after the temporal artery biopsy results?

A

BEFORE

53
Q

what can develop from polyarteritis nodosa?

A

microaneurysms

aneurysms

54
Q

areas often affected by polyarteritis nodosa?

A

skin
gut
kidneys

55
Q

which condition is associated with late onset asthma

A

eGPA

56
Q

what small vessel vasculitis commonly presents with necrotising glomerulonephritis?

A

GPA

MPA

57
Q

least common small vessel vasculitis?

A

MPA

58
Q

what increases risk of GPA?

A

smoking
silica
drugs
staph aureus carriage in nose

59
Q

typical age of GPA onset?

A

35-55

60
Q

clinical symptoms of gpa?

A

ent symptoms plus chest symptoms, vasculitis rash, kidney involvement

61
Q

histological features of gpa?

A

granulomatous inflam in arterial wall

62
Q

resp symptoms of gpa?

A
pulmonary infiltrates
cough
haemoptysis
diffuse alveolar haemorrhage
cavitating granulomas on cxr
63
Q

nervous symptoms of gpa?

A

mononeuritis multiplex
sensorimotor polyneuropathy
cranial nerve palsy

64
Q

ocular symptoms of gpa?

A
conjunctivitis
episcleritis
uveitis
optic nerve vascultis
retinal artery occlusion
proptosis
65
Q

what symptoms of eGPA are not present in GPA?

A

late onset asthma
high eosinophil count
less ENT features

66
Q

where is a vasculitis rash most commonly found?

A

legs and feet

67
Q

which small vessel vasculitis is associated with cANCA?

A

GPA

68
Q

which small vessel vascultiis is associated with pANCA?

A

MPA

EGPA

69
Q

does cANCA or pANCA vary with disease activity?

A

both do

70
Q

is cANCA or pANCA more specific?

A

cANCA

71
Q

when would vasculitis be classed as severe?

A

when renal creatinine >500

or organs are threatened

72
Q

treatment for mild AAV?

A

methotrexate

steroids

73
Q

treatment for severe AAV?

A
  1. IV cyclophosphamide and steroids

plasma exchange if creatinine >500

74
Q

what should you give after severe vasculitis has calmed down?

A

azathioprine

methotrexate

75
Q

what should you give if severe AAV treatment isnt working?

A

IV immunoglobulins

rituximab

76
Q

what antibody mediates henoch schonelin purpura?

A

IgA

77
Q

who gets HSP?

A

children aged 2-11

78
Q

what systemic areas are affected in HSP?

A

GI
kidneys
joints

79
Q

what can HSP be triggered by?

A

URTI
pharyngeal infection
GI infection
group A strep

80
Q

how long before HSP are the triggers usually?

A

1-3 weeks before

81
Q

clinical presentation of HSP?

A
purpuric rash over bum and lower limb
colicky abdo pain
bloody diarrhoea
joint pain/swelling
renal involvement
82
Q

tx for HSP?

A

self limiting

fixes itself within 8 weeks

83
Q

investigations for HSP?

A

URINALYSIS