Week 2 - Vasculitis Flashcards

1
Q

what is vasculitis?

A

inflammation of the blood vessels

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2
Q

what can vasculitis cause in blood vessels?

A

vessel wall thickening
stenosis
occlusion subsequent ischaemia

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3
Q

what is large vessel vasculitis?

A

primary vasculitis that causes granulomatous inflammation predominantly of the aorta and its major branches

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4
Q

what are the 2 categories of large vessel vasculitis?

A
temporal (giant cell) arteritis (GCA)
Takayasu arteritis (TA)
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5
Q

how do GCA and TA differ?

A

age of onset

  • GCA = <50
  • TA = >50
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6
Q

where is TA more common, east or west?

A

east

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7
Q

who does TA most commonly affect?

A

young women in 2nd and third decade of life

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8
Q

where does GCA usually effect?

A

temporal arteries but may also or only affect the large vessels

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9
Q

what are some early features of large vessel vasculitis?

A
low grade fever
malaise
night sweats
weight loss
arthralgia
fatigue
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10
Q

what are some later features of large vessel vasculitis?

A

claudication in upper and lower limbs
vascular stenosis
aneurysms

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11
Q

what are some examination findings of late large vessel vasculitis?

A

reduced pulses

bruits

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12
Q

large vessel vasculitis blood tests?

A

elevated CRP, ESR and PV

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13
Q

how can imaging be used to detect large vessel vasculitis?

A

MR angiography can detect thickened vessel walls and stenosis
PET CT can show increased metabolic activity in large vessels

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14
Q

what is the main treatment for large vessel vasculitis?

A

corticosteroids (prednisolone)

steroids sparing agents like methotrexate and azathioprine can be added

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15
Q

what does of corticosteroid is used in large vessel vasculitis?

A

start at 40-60 depending on severity and reduce over 18 months to 2 years

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16
Q

what are the 2 types of small vessel vasculitis?

A

ANCA positive

ANCA negative

17
Q

what are the 3 types of ANCA associated small vessel vasculitis?

A

granulomatosis with polyangiitis
microscopic polyangiitis
eosinophilic granulomatosis with polyangitis

18
Q

features of each ANCA associated small vessel vasculitis?

A
GPA = granulomas
EGPA = granulomas, asthma, eosinophila
MPA = no granuloma
19
Q

what are some common presenting features in ANCA associated vasculitis?

A
fever and weight loss
purpuric vasculitic rash
arthralgia/arthritis
mononeuritis multiplex
glomerulonephritis
lung opacities
20
Q

ENT symptoms of GPA?

A

nose bleeds, deafness, recurrent sinusitis, nasal crusting, nose collapse over time

21
Q

resp symptoms of GPA?

A

haemoptysis

cavitating lesions on X ray

22
Q

what antibodies are associated with GPA?

A

cANCA and PR3

23
Q

what characterises EGPA?

A

late onset asthma
rhinitis
raised peripheral blood eosinophil count

24
Q

can neurological symptoms occur with EGPA?

A

yes

mononeuritis multiplex

25
Q

what antibodies are associated with EGPA?

A

pANCA

MPO

26
Q

what mediates ANCA associated vasculitis?

A

immune complexes

27
Q

what is the most important complication of microscopic polyangiitis?

A

glomerulonephritis

occurs in up to 90% of patients

28
Q

can ANCA be relied on for diagnosis?

A

no

its negative in in come ANCA associated vasculitis cases

29
Q

how is AAV investigated?

A
bloods
auto antibodies (ANA)
urinalysis (renal vasculitis)
CXR
Biopsy of affected area
30
Q

what is seen on blood tests in AAV?

A

anaemia if chronic
elevated CRP, ESR and PV
U&Es check renal involvement

31
Q

how is AAV managed?

A

most need IV steroids and cyclophosphamide

32
Q

what is Henoch schonlein purpura?

A

acute IgA mediated disorder causing generalised vasculitis of small vessels in skin, GI tract, kidneys, joints, lungs and sometimes CNS

33
Q

who does Henoch schonlein purpura usually effect?

A

children

few weeks after URTI

34
Q

what are the common symptoms of Henoch schonlein purpura?

A

purpuric rash over buttocks and lower limbs
abdo pain
vomiting
joint pain

35
Q

how is Henoch schonlein purpura treated?

A

usually self limiting over weeks to months