Vasculitis Flashcards
pathogenesis behind primary vasculitis
idiopathic inflammatory response against vessel walls
can be AI
pathogenesis of secondary vasculitis
trigger of infection/drug/toxin
or part of another inflammatory disorder/ cancer
ANCA associated small vessel vasculitis egs (3)
microscopic polyangiitis
GPA
EGPA
immune complex small vessel vasculitis eg.s (3)
cryoglobulinaemic vasculitis
Henoch Schonlein (IgA)
hypocomplementemic urticarial vasculitis (Anti - C1q vasculitis)
Takayasu arteritis = _____ infiltratioin of vessel walls
age
M:F
ethnicity
granulomatous
<40yo
M
5 possible presenting features of large vessels vasculitis (Takayasu + GCA)
bruit, commonly carotid bp difference between extremities claudication carotodynia/vessel tenderness hbp
50% of GCA have ___, 15% of ___ develop GCA
PMR
scalp tenderness, jaw claudication, prominent temporal vessels with reduced pulsation =
temporal arteritis
Ix for large vessel vasculitis (takayasu + GCA) and results
ESR,PV and CRP high
temporal artery biopsy
MR angiography / PET CT (see vessel wall thickening/stenosis/aneurysm or increased metabolic activity)
dose and which steroid for -ve/+ve visual changes in GCA
taper over ___
if can’t reduce then add in ___
-ve = 40mg prednisolone
+ve = 60mg prednisolone
18months
methotrexate/azathioprine
medium vessel vasculitis that affects <5yos and may lead to coronary artery aneurysm
Kawasaki
necrotising inflammatory lesions at bifurcations => microaneurysm and aneurysms
often in skin, gut and kidneys
ass with hep __
Polyarteritis nodosa
hepB
typical age for GPA
35-55yo
s+s of GPA
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
saddle nose =
GPA
pulmonary s+s of GPA =
infiltrates cough haemoptysis diffuse alveolar haemorrhage CXR = cavitating nodules
EGPA 2 distinguishing features
late onset asthma
high eosinophil count
EGPA: ACR criteria (>=4) for diagnosis =
asthma eosinophilia >10% in peripheral blood histology = vasculitis with extravascular eosinos paranasal sinusitis pulmonary infiltrates mononeuritis multiplex/polyneuropathy
ANCAs are found in ___ of ___ granulocytes - seen with ___
cytoplasm
neutrophil
immunofluorescence
microscopic polyangiitis is associated with _ANCA
70-90% are __ +ve
10-30% are ___ +ve
pANCA
70-90 = MPO
10-30 = PR3
GPA is ass with _ANCA
70-90% ass with __
5-10% with __
cANCA
70-90 = PR3
5-10 = MPO
EGPA is ass with _ANCA
<2% ass with __
30-70% ass with __
pANCA
2 = PR3
30-70 = MPO
only way to confirm presence of glomerulonephritis
renal biopsy
management of medium vessel vasculitis
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
management of medium vessel vasculitis if localised/ early systemic
methotrexate/azxathioprine + steroids
management of medium vessel vasculitis if generalised/systemic
generalised/systemic = cyclophosphamine + steroids (1st line) / rituximab +steroids ; plasma exchange if creatinine >500
may lead to AZT alternatives - methotrexate, mycophenolate mofetil
management of medium vessel vasculitis if refrective
IV Ig, rituximab
acute IgA mediated generalised vasculitis of small vessels in skni, gut, kidneys, joints (rarely - CNS + lungs)
Henoch Schonlein
2 age peaks in Henoch Schonlein purpura
75% 2-11 yo
elderly after infection
> 75% of Henoch Schonlein purpura ptnts have a preceding __
URTI, pharyngeal/GI infection
most common = strep pyogenes 1-3 wks before onset
5 presenting features of Henoch Schonlein purpura
purpuric rash (buttocks and lower limb) colic bloody diarrhoea arthralgia +/- swelling 50% have renal involvement
management of Henoch Schonlein purpura
self limiting in 8 wks
relapses can occure for months/yrs
urinalysis in case of renal involvement
