Vasculitis Flashcards

(83 cards)

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
who gets kawasaki disease?
children under 5
26
most common arteries affected by kawasaki disease? why is this relevant?
coronary; can develop aneurysm
27
where does polyarteritis nodosa typically affect?
arteries at vessel bifurcations
28
which vasculitis is associated with hep b
medium vessel
29
which syndromes should you consider when the respiratory tract is affected?
GPA | eGPA
30
who tends to get gpa?
people of northern european descent in their middle age
31
another name for wegeners?
granulomatosis with polyangiitis
32
another name for churg strauss syndrome
eosinophilic granulomatosis with polyangiitis
33
what would a chest x ray show for gpa?
nodules fixed infiltrates cavities
34
ENT features of gpa
sinusitis nasal crusting mouth ulcers saddle nose
35
main difference between churg strauss and wegeners
get late onset asthma and high eosinophil count in churg strauss
36
which condition is cANCA raised in?
granulomatous polyangiitis/ wegeners
37
which condition is pANCA raised in?
eosinophilic granulomatous polyangiitis
38
is anti-PR3 associated with gpa or egpa
GPA
39
management of ANCA +ve vasculitis
early- methotrexate + steroids | systemic - cyclophosphamide +steroids OR 2nd line rituximab + steroids
40
differentials other than vasculitis
chronic infection | cancer
41
areas that GCA can affect?
temporal artery aorta subclavian artery
42
areas that takayasu arteritis affects?
aorta
43
why can someone with GCA get claudication symptoms?
if subclavian/aorta are involved and they are doing repetitive activity
44
what kind of inflammation exists in large vessel vascultis?
granulomatous
45
presenting features of large vessel vasculitis?
``` bruit blood pressure difference of extremities claudication carotodynia hypertension ```
46
what percentage of people with GCA have PMR?
50%
47
what percentage of people with PMR have GCA?
15%
48
symptoms of GCA?
unilateral temporal headache scalp tenerness jaw claudication
49
when would you do an MR angiogram or PET CT of someone with GCA?
if vessels other than the temporal artery are involved
50
what would an MR angiogram show?
vessel wall thickening/stenosis/aneurysm
51
how does a PET CT help in someone with GCA?
shows where metabolically active cells eg inflamed cells are
52
should you start steroids for GCA before or after the temporal artery biopsy results?
BEFORE
53
what can develop from polyarteritis nodosa?
microaneurysms | aneurysms
54
areas often affected by polyarteritis nodosa?
skin gut kidneys
55
which condition is associated with late onset asthma
eGPA
56
what small vessel vasculitis commonly presents with necrotising glomerulonephritis?
GPA | MPA
57
least common small vessel vasculitis?
MPA
58
what increases risk of GPA?
smoking silica drugs staph aureus carriage in nose
59
typical age of GPA onset?
35-55
60
clinical symptoms of gpa?
ent symptoms plus chest symptoms, vasculitis rash, kidney involvement
61
histological features of gpa?
granulomatous inflam in arterial wall
62
resp symptoms of gpa?
``` pulmonary infiltrates cough haemoptysis diffuse alveolar haemorrhage cavitating granulomas on cxr ```
63
nervous symptoms of gpa?
mononeuritis multiplex sensorimotor polyneuropathy cranial nerve palsy
64
ocular symptoms of gpa?
``` conjunctivitis episcleritis uveitis optic nerve vascultis retinal artery occlusion proptosis ```
65
what symptoms of eGPA are not present in GPA?
late onset asthma high eosinophil count less ENT features
66
where is a vasculitis rash most commonly found?
legs and feet
67
which small vessel vasculitis is associated with cANCA?
GPA
68
which small vessel vascultiis is associated with pANCA?
MPA | EGPA
69
does cANCA or pANCA vary with disease activity?
both do
70
is cANCA or pANCA more specific?
cANCA
71
when would vasculitis be classed as severe?
when renal creatinine >500 | or organs are threatened
72
treatment for mild AAV?
methotrexate | steroids
73
treatment for severe AAV?
1. IV cyclophosphamide and steroids | plasma exchange if creatinine >500
74
what should you give after severe vasculitis has calmed down?
azathioprine | methotrexate
75
what should you give if severe AAV treatment isnt working?
IV immunoglobulins | rituximab
76
what antibody mediates henoch schonelin purpura?
IgA
77
who gets HSP?
children aged 2-11
78
what systemic areas are affected in HSP?
GI kidneys joints
79
what can HSP be triggered by?
URTI pharyngeal infection GI infection group A strep
80
how long before HSP are the triggers usually?
1-3 weeks before
81
clinical presentation of HSP?
``` purpuric rash over bum and lower limb colicky abdo pain bloody diarrhoea joint pain/swelling renal involvement ```
82
tx for HSP?
self limiting | fixes itself within 8 weeks
83
investigations for HSP?
URINALYSIS