Week 2 - Vasculitis Flashcards
what is vasculitis?
inflammation of the blood vessels
what can vasculitis cause in blood vessels?
vessel wall thickening
stenosis
occlusion subsequent ischaemia
what is large vessel vasculitis?
primary vasculitis that causes granulomatous inflammation predominantly of the aorta and its major branches
what are the 2 categories of large vessel vasculitis?
temporal (giant cell) arteritis (GCA) Takayasu arteritis (TA)
how do GCA and TA differ?
age of onset
- GCA = <50
- TA = >50
where is TA more common, east or west?
east
who does TA most commonly affect?
young women in 2nd and third decade of life
where does GCA usually effect?
temporal arteries but may also or only affect the large vessels
what are some early features of large vessel vasculitis?
low grade fever malaise night sweats weight loss arthralgia fatigue
what are some later features of large vessel vasculitis?
claudication in upper and lower limbs
vascular stenosis
aneurysms
what are some examination findings of late large vessel vasculitis?
reduced pulses
bruits
large vessel vasculitis blood tests?
elevated CRP, ESR and PV
how can imaging be used to detect large vessel vasculitis?
MR angiography can detect thickened vessel walls and stenosis
PET CT can show increased metabolic activity in large vessels
what is the main treatment for large vessel vasculitis?
corticosteroids (prednisolone)
steroids sparing agents like methotrexate and azathioprine can be added
what does of corticosteroid is used in large vessel vasculitis?
start at 40-60 depending on severity and reduce over 18 months to 2 years
what are the 2 types of small vessel vasculitis?
ANCA positive
ANCA negative
what are the 3 types of ANCA associated small vessel vasculitis?
granulomatosis with polyangiitis
microscopic polyangiitis
eosinophilic granulomatosis with polyangitis
features of each ANCA associated small vessel vasculitis?
GPA = granulomas EGPA = granulomas, asthma, eosinophila MPA = no granuloma
what are some common presenting features in ANCA associated vasculitis?
fever and weight loss purpuric vasculitic rash arthralgia/arthritis mononeuritis multiplex glomerulonephritis lung opacities
ENT symptoms of GPA?
nose bleeds, deafness, recurrent sinusitis, nasal crusting, nose collapse over time
resp symptoms of GPA?
haemoptysis
cavitating lesions on X ray
what antibodies are associated with GPA?
cANCA and PR3
what characterises EGPA?
late onset asthma
rhinitis
raised peripheral blood eosinophil count
can neurological symptoms occur with EGPA?
yes
mononeuritis multiplex
what antibodies are associated with EGPA?
pANCA
MPO
what mediates ANCA associated vasculitis?
immune complexes
what is the most important complication of microscopic polyangiitis?
glomerulonephritis
occurs in up to 90% of patients
can ANCA be relied on for diagnosis?
no
its negative in in come ANCA associated vasculitis cases
how is AAV investigated?
bloods auto antibodies (ANA) urinalysis (renal vasculitis) CXR Biopsy of affected area
what is seen on blood tests in AAV?
anaemia if chronic
elevated CRP, ESR and PV
U&Es check renal involvement
how is AAV managed?
most need IV steroids and cyclophosphamide
what is Henoch schonlein purpura?
acute IgA mediated disorder causing generalised vasculitis of small vessels in skin, GI tract, kidneys, joints, lungs and sometimes CNS
who does Henoch schonlein purpura usually effect?
children
few weeks after URTI
what are the common symptoms of Henoch schonlein purpura?
purpuric rash over buttocks and lower limbs
abdo pain
vomiting
joint pain
how is Henoch schonlein purpura treated?
usually self limiting over weeks to months
