Vasculitis Flashcards
define vasculitis
inflammation of blood vessels
may result in vessel wall thickening, stenosis and occlusion with subsequent ischaemia
why does the clinical presentation vary
according to the histological type of inflammation
size of involved blood vessel segment
distribution of involved vessels
how is it classified
Chapel Hill Consensus
name the 2 large vessel vaculitis
giant cell arthritis
takayasu arthritis
large vessel vasculitis
applies to primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches
what age does large vessel vasculitis occur in
GCA - >50 (polymyalgia rheumatica)
Takayasu <50 (women in 20s and 30s)
where does GCA typically affect
temporal arteries
who does TA most commonly affect
young women in their second and third decades of life
clinical presentation of large vessel vasculitis
early non specific features eg fever, weight loss, night sweats, malaise, arthralgia and fatigue
following this claudication symptoms
what can happen if large vessel vasculitis is untreated
vascular stenosis and aneurysms
results in reduced pulses and bruits
inflammatory markers for large vessel vasculitis
CRP, ESV and PV elevated
what imaging is used in large vessel vasculitis
MR angiography can detect thickened walls and stenosis
PE CT shows inc metabolic activity in the larger vessels
treatment of large vessel vasculitis
corticosteroids - starting a 40/60mg prednisolone and gradually reducing
steroid sparing agents such as methotrexate and azathioprine may be added
how is small to medium vessel vasculitis divided
into ANCA positive and negative
ANCA positive
no granuloma present
microscopic polyangiitis
ANCA positive
granuloma present
asthma an eosinophil present
churg strauss
ANCA positive
granuloma present
asthma an eosinophil not present
wegeners granulomatosis
non ANCA
IgA dominant immune deposit
henoch-schlnein purpura
non ANCA
not IgA dominant immune deposit
serum cryoglobulin
cryoglobulinemia
non ANCA
not IgA dominant immune deposit
not serum cryoglobulin
other
what features do many small/medium vessel vasculitis conditions share
fever and weight loss
raised non planching purpuric rash
arthraliga/arthritis
mononeuritis multiplex
glomerulonephritis
lung opacities on X ray
what is the vasculitis rash like
raised non-blanching purpuric rash
GPA/Wegeners
ENT symptoms common - nose bleed, deafness, recurrent sinusitis and nasal crusting
respiratory symptoms - haemoptysis and cavitating lesions on X ray
renal failure
what can happen to the nose over time in GPA
recurrent sinusitis and nasal crusting can lead to collapse of the nose
what can be seen in the lungs in GPA
cavitating opacities
what proteins is GPA associated with
cANCA and PR3
EGPA (Churg Strauss) symptoms
late onset asthma, rhinitis and raised peripheral blood eosinophil count
what neurological symptoms are seen in EGPA
mononeuritis multiplex
what type of ANCA for EGPA
pANCA (30-70%)
what is the most important complication of microscopic polyangiitis
(no granulomatous inflammation)
glomerulonephritis - up to 90% patients
what antibody is present in MPA
pANCA (MPO) in 70-90%
which ANCA associated vasculitis is upper airway disease most common in
GPA
then EGPA
how reliable is ANCA
is negative in a proportion of all these conditions so cannot be relied upon for a diagnosis
investigations for small/medium vessel vasculitis
ESR, PV, CRP raised
anaemic of chronic disease is common
U and E for renal involvement
ANCA
urinalysis (looking for renal vasculitis)
CXR
biopsy of affected area
management of ANCA associated vasculitis
most cases of ANCA associated vasculitis require treatment with IV steroids and cyclophosphamide due to their agressive disease course
there are some situations eg GPA localised in the nose where other options would be considered
Henoch-Schonlein purpura
acute IgA mediated disorder characterised by generalised vasculitis involving the small vessels of the skin, the GI tract, the kidneys, joints, and the lungs and CNS system rarely
who does Henoch-Schonlein purpura commonly affect
children
what commonly predates Henoch-Schonlein purpura symptoms by a few weeks
history of URT infection
common symptoms of Henoch-Schonlein purpura
purpuric rash over the buttocks and lower limbs, abdominal pain and vomiting and joint pain
treatment of Henoch-Schonlein purpura
usually self limiting and doesnt require specific treatment
usually settles over the course of weeks to monthhs
Cryoglobulinaemia
symptoms of peripheral rash, ulceration and Raynaud’s are typical
