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MSKJaz > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

pathogenesis behind primary vasculitis

A

idiopathic inflammatory response against vessel walls

can be AI

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2
Q

pathogenesis of secondary vasculitis

A

trigger of infection/drug/toxin

or part of another inflammatory disorder/ cancer

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3
Q

ANCA associated small vessel vasculitis egs (3)

A

microscopic polyangiitis
GPA
EGPA

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4
Q

immune complex small vessel vasculitis eg.s (3)

A

cryoglobulinaemic vasculitis
Henoch Schonlein (IgA)
hypocomplementemic urticarial vasculitis (Anti - C1q vasculitis)

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5
Q

Takayasu arteritis = _____ infiltratioin of vessel walls
age
M:F
ethnicity

A

granulomatous
<40yo
M

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6
Q

5 possible presenting features of large vessels vasculitis (Takayasu + GCA)

A 
bruit, commonly carotid
bp difference between extremities
claudication
carotodynia/vessel tenderness
hbp
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7
Q

50% of GCA have ___, 15% of ___ develop GCA

A

PMR

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8
Q

scalp tenderness, jaw claudication, prominent temporal vessels with reduced pulsation =

A

temporal arteritis

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9
Q

Ix for large vessel vasculitis (takayasu + GCA) and results

A

ESR,PV and CRP high
temporal artery biopsy
MR angiography / PET CT (see vessel wall thickening/stenosis/aneurysm or increased metabolic activity)

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10
Q

dose and which steroid for -ve/+ve visual changes in GCA
taper over ___
if can’t reduce then add in ___

A

-ve = 40mg prednisolone
+ve = 60mg prednisolone
18months
methotrexate/azathioprine

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11
Q

medium vessel vasculitis that affects <5yos and may lead to coronary artery aneurysm

A

Kawasaki

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12
Q

necrotising inflammatory lesions at bifurcations => microaneurysm and aneurysms
often in skin, gut and kidneys
ass with hep __

A

Polyarteritis nodosa

hepB

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13
Q

typical age for GPA

A

35-55yo

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14
Q

s+s of GPA

A 
constitutionals + arthralgia
nasal/ oral inflammation
sinusitis, nasal crusting, epistaxis, mouth ulcers
saddle nose (due to cartilage ischaemia)
sensorineural deafness
Otitis media and deaf
pulmonary, necrotising glomerulonephritis, mononeuritis multiplex
eye inflammation
palpable purpura lower leg
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15
Q

saddle nose =

A

GPA

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16
Q

pulmonary s+s of GPA =

A 
infiltrates
cough 
haemoptysis
diffuse alveolar haemorrhage
CXR = cavitating nodules
17
Q

EGPA 2 distinguishing features

A

late onset asthma

high eosinophil count

18
Q

EGPA: ACR criteria (>=4) for diagnosis =

A 
asthma
eosinophilia >10% in peripheral blood
histology = vasculitis with extravascular eosinos
paranasal sinusitis
pulmonary infiltrates
mononeuritis multiplex/polyneuropathy
19
Q

ANCAs are found in ___ of ___ granulocytes - seen with ___

A

cytoplasm
neutrophil
immunofluorescence

20
Q

microscopic polyangiitis is associated with _ANCA
70-90% are __ +ve
10-30% are ___ +ve

A

pANCA
70-90 = MPO
10-30 = PR3

21
Q

GPA is ass with _ANCA
70-90% ass with __
5-10% with __

A

cANCA
70-90 = PR3
5-10 = MPO

22
Q

EGPA is ass with _ANCA
<2% ass with __
30-70% ass with __

A

pANCA
2 = PR3
30-70 = MPO

23
Q

only way to confirm presence of glomerulonephritis

A

renal biopsy

24
Q

management of medium vessel vasculitis

A

if localised/early systemic = methotrexate/azxathioprine + steroids
generalised/systemic = cyclophosphamine + steroids (1st line) / rituximab +steroids ; plasma exchange if creatinine >500
may lead to AZT alternatives - methotrexate, mycophenolate mofetil
refractory = IV Ig, rituximab

25
Q

management of medium vessel vasculitis if localised/ early systemic

A

methotrexate/azxathioprine + steroids

26
Q

management of medium vessel vasculitis if generalised/systemic

A

generalised/systemic = cyclophosphamine + steroids (1st line) / rituximab +steroids ; plasma exchange if creatinine >500
may lead to AZT alternatives - methotrexate, mycophenolate mofetil

27
Q

management of medium vessel vasculitis if refrective

A

IV Ig, rituximab

28
Q

acute IgA mediated generalised vasculitis of small vessels in skni, gut, kidneys, joints (rarely - CNS + lungs)

A

Henoch Schonlein

29
Q

2 age peaks in Henoch Schonlein purpura

A

75% 2-11 yo

elderly after infection

30
Q

> 75% of Henoch Schonlein purpura ptnts have a preceding __

A

URTI, pharyngeal/GI infection

most common = strep pyogenes 1-3 wks before onset

31
Q

5 presenting features of Henoch Schonlein purpura

A 
purpuric rash (buttocks and lower limb)
colic
bloody diarrhoea
arthralgia +/- swelling
50% have renal involvement
32
Q

management of Henoch Schonlein purpura

A

self limiting in 8 wks
relapses can occure for months/yrs
urinalysis in case of renal involvement

MSKJaz (28 decks)

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