Vasculitis Flashcards
what is vasculitis
inflammation of blood vessels,
often with ischaemia, necrosis and organ inflammation
what blood vessels can vasculitis affect
all of them
what level should eosinophils not exceed?
1
which size of vessel has the worst prognosis when affected by vasculitis
small vessels
what is primary vasculitis
vasculitis caused by an inflammatory response that targets the blood vessels with no known cause
what is secondary vasculitis
vasculitis triggered by an infection, drug, toxin or cancer
what do activated T cells do to the blood vessels?
promote inflammation, granuloma formation, macrophage activation and differentiation
what activates the T cells
inflammatory cytokines released from dendritics
name the 2 forms of large vessel vasculitis
takayasu arteritis
giant cell arteritis
name 2 forms of medium vessel vasculitis
polyareritis nodosa
kawasaki disease
what 2 categories is small vessel vasculitis divided into?
immune complex
ANCA associated
which small vessel vasculitis category is polyangiitis associated with?
ANCA-associated
ANCA positive small vessel vasculitis with granulomas, asthma and eosinophilia?
egpa
ANCA positive small vessel vasculitis with granulomas only?
gpa
ANCA negative small vessel vasculitis with IgA dominant immune deposit
henoch schonlein purpura
ANCA negative small vessel vasculitis with no IgA and no serum ceyoglobulin?
other ANCA negative vasculitis eg IBD vasculitis
ANCA negative small vessel vasculitis with no IgA immune deposit and has serum cryoglobulin present
cryoglobulinaemia
what systemic symptoms are common to all vasculitides
fever
malaise
weight loss
fatigue
who tends to get takayasu arteritis
asian women under 40
who tends to get giant cell arteritis
elderly people
presenting features of giant cell arteritis?
carotid bruits
claudication
HT
BP difference in extremities
common symptoms of temporal arteritis
polymyalgia rheumatica
unilateral temporal headache
scalp tenderness
jaw claudication
investigations for temporal arteritis
ESR plasma viscosity CRP temporal artery biopsy PET CT MR angiogram
treatment for temporal arteritis
40-60mg prednisolone
consider methotrexate or azathioprine
who gets kawasaki disease?
children under 5
most common arteries affected by kawasaki disease? why is this relevant?
coronary; can develop aneurysm
where does polyarteritis nodosa typically affect?
arteries at vessel bifurcations
which vasculitis is associated with hep b
medium vessel
which syndromes should you consider when the respiratory tract is affected?
GPA
eGPA
who tends to get gpa?
people of northern european descent in their middle age
another name for wegeners?
granulomatosis with polyangiitis
another name for churg strauss syndrome
eosinophilic granulomatosis with polyangiitis
what would a chest x ray show for gpa?
nodules
fixed infiltrates
cavities
ENT features of gpa
sinusitis
nasal crusting
mouth ulcers
saddle nose
main difference between churg strauss and wegeners
get late onset asthma and high eosinophil count in churg strauss
which condition is cANCA raised in?
granulomatous polyangiitis/ wegeners
which condition is pANCA raised in?
eosinophilic granulomatous polyangiitis
is anti-PR3 associated with gpa or egpa
GPA
management of ANCA +ve vasculitis
early- methotrexate + steroids
systemic - cyclophosphamide +steroids OR 2nd line rituximab + steroids
differentials other than vasculitis
chronic infection
cancer
areas that GCA can affect?
temporal artery
aorta
subclavian artery
areas that takayasu arteritis affects?
aorta
why can someone with GCA get claudication symptoms?
if subclavian/aorta are involved and they are doing repetitive activity
what kind of inflammation exists in large vessel vascultis?
granulomatous
presenting features of large vessel vasculitis?
bruit blood pressure difference of extremities claudication carotodynia hypertension
what percentage of people with GCA have PMR?
50%
what percentage of people with PMR have GCA?
15%
symptoms of GCA?
unilateral temporal headache
scalp tenerness
jaw claudication
when would you do an MR angiogram or PET CT of someone with GCA?
if vessels other than the temporal artery are involved
what would an MR angiogram show?
vessel wall thickening/stenosis/aneurysm
how does a PET CT help in someone with GCA?
shows where metabolically active cells eg inflamed cells are
should you start steroids for GCA before or after the temporal artery biopsy results?
BEFORE
what can develop from polyarteritis nodosa?
microaneurysms
aneurysms
areas often affected by polyarteritis nodosa?
skin
gut
kidneys
which condition is associated with late onset asthma
eGPA
what small vessel vasculitis commonly presents with necrotising glomerulonephritis?
GPA
MPA
least common small vessel vasculitis?
MPA
what increases risk of GPA?
smoking
silica
drugs
staph aureus carriage in nose
typical age of GPA onset?
35-55
clinical symptoms of gpa?
ent symptoms plus chest symptoms, vasculitis rash, kidney involvement
histological features of gpa?
granulomatous inflam in arterial wall
resp symptoms of gpa?
pulmonary infiltrates cough haemoptysis diffuse alveolar haemorrhage cavitating granulomas on cxr
nervous symptoms of gpa?
mononeuritis multiplex
sensorimotor polyneuropathy
cranial nerve palsy
ocular symptoms of gpa?
conjunctivitis episcleritis uveitis optic nerve vascultis retinal artery occlusion proptosis
what symptoms of eGPA are not present in GPA?
late onset asthma
high eosinophil count
less ENT features
where is a vasculitis rash most commonly found?
legs and feet
which small vessel vasculitis is associated with cANCA?
GPA
which small vessel vascultiis is associated with pANCA?
MPA
EGPA
does cANCA or pANCA vary with disease activity?
both do
is cANCA or pANCA more specific?
cANCA
when would vasculitis be classed as severe?
when renal creatinine >500
or organs are threatened
treatment for mild AAV?
methotrexate
steroids
treatment for severe AAV?
- IV cyclophosphamide and steroids
plasma exchange if creatinine >500
what should you give after severe vasculitis has calmed down?
azathioprine
methotrexate
what should you give if severe AAV treatment isnt working?
IV immunoglobulins
rituximab
what antibody mediates henoch schonelin purpura?
IgA
who gets HSP?
children aged 2-11
what systemic areas are affected in HSP?
GI
kidneys
joints
what can HSP be triggered by?
URTI
pharyngeal infection
GI infection
group A strep
how long before HSP are the triggers usually?
1-3 weeks before
clinical presentation of HSP?
purpuric rash over bum and lower limb colicky abdo pain bloody diarrhoea joint pain/swelling renal involvement
tx for HSP?
self limiting
fixes itself within 8 weeks
investigations for HSP?
URINALYSIS
