Vasculitis Flashcards
What is the most common form of systemic vasculitis in adults?
giant cell arteritis
what histologically is seen in giant cell arteritis?
transmural inflammation (involving intima, media and adventita) as well as patchy granulomatous infiltration of lymphocytes, macrophages and multinucleated giant cells
why can giant cell arteritis cause distal ischaemia?
the artery wall becomes thickened due to the inflammation. This causes luminal narrowing.
what should you always consider in a patient over 50 y/o with a new onset headache and raised CRP/PV/ESR?
giant cell arteritis
what artery is commonly affected in giant cell arteritis?
temporal artery
what is the most serious consequence of giant cell arteritis in the temporal artery?
permanent visual impairement
describe the headache commonly seen in patients with temporal arteritis?
continuous and located in the temporal or occipital areas
focal tenderness on direct palpation
why can patient with temporal arteritis experience jaw claudication?
ischamia of the maxillary artery
what is jaw claudication?
jaw fatigue or discomfort during chewing or prolonged speaking
what is the most definitive test for suspected giant cell arteritis?
temporal artery biopsy
compare specificity to sensitivity in terms of a temporal artery biopsy for GCA?
high specificity
low sensitivity
why does temporal artery biopsy have a low sensitivity for GCA?
because there is patchy involvement- some segments of temporal artery might be normal
what is the treatment for temporal arteritis with visual symptoms?
60mg prednisolone tapered over 2 years
what is the treatment for temoral arteritis without visual symptoms?
40mg prednisolone tapered over 2 years
when should treatment with steroids be started for a patient with suspected temporal arteritis?
as soon as diagnosis is suspected
don’t wait for biopsy results
what is large vessel vasculitis?
primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its branches
what are the 2 major categories of large vessel vasculitis?
Takayasu arteritis
Giant cell arteritis
who tends to get takayasu?
young women
mainly in east asian countries
what are the clinical signs of large vessel vasculitis?
reduced pulses
bruits
what is the mainstay treatment of large vessel vasculitis?
40-60mg prednisolone
tapering doses
steroid sparing agents eg methotrexate and azathioprine may be used
what are the 2 main classes of small vessel vasculitis?
ANCA-associated
Non-ANCA- associated
within ANCA-associated small vessel vasculitis, what is the further subdivision?
granulomas - GPA or EGPA
no granulomas- MPA
What are the main differences between EGPA and GPA?
EGPA- eosinophilia, late onset asthma, cANCA
GPA- common ENT symptoms, pANCA
what is the most important complication of microscopic polyangitis?
glomerulonephritis
what is the management of ANCA-associated vasculitis?
IV steroids
cyclophosphamide
what is Henoch-Schonlein purpura?
an acute IgA mediated disorder which commonly affects children after a history of a upper respiratory tract infection
what is the treatment of Henoch schonein purpura?
self-limiting
what is vasculitis?
inflammation of blood vessels, often with ischamia, necrosis and organ inflammation
why can vasculitis lead to blood clot formation?
lumen becomes narrower and irregular which promotes blood clots formting
what are the 2 main classes of medium vessel vaculitis-?
polyarteritis nodosa
kawasaki disease
what are both takayasu arteritis and giant cell arteritis characterised by?
granulomatous infiltration of the walls of large vessels
why are granulomas generally formed in the body?
to wall off a ‘threat’ from the body
one that can’t be expelled
what type of cells from a granuloma merge together to form giant cells?
macrophages
giant cell arteritis
what are the main clinical findings of large vessel vasculitis?
- bruit (usually carotid)
- blood pressure difference of extremities
- vessel tenderness (usually unilateral)
- hypertension
what clinical sign may indicate temporal arteritis?
prominent temporal arteries
reduced pulsation
who tends to get kawasaki disease?
children under 5
aneurysms to what arteries are a significant risk in kawaski disease?
coronary arteries
when do you choose to give treatment in kawaski disease?
when coronary arteries are affected
if they aren’t affected this condition will subside without treatment
where in the blood does polyarteritis nodosa tend to affect?
birfucations
what can result from the necrotising inflammatory lesions caused by polyarteritis nodosa?
microaneurysm and aneurysm formation
what viral disease is polyarteritis nodosa associated with?
hepatitis B
what is the new name of Wegner’s granulomatosis?
granulomatosis with polyangiitis (GPA)
what is the new name of Churg-Strauss syndrome?
Eosinophilic granulomatosis with polyangitiis (EGPA)
what respiratory disease to patients with eosinophilc granulomatosis with polyangitiis frequently get?
late-onset asthma
what is a ‘saddle nose’?
collapse of nasal cartilage
what causes a saddle nose?
collapse of nasal cartilage caused by cartilage ischaemia
ENT features such as this present in GPA
what are the main 2 cutaneous features of small vessel vasculitis?
ulcers palpable purpura (ie raised purpura)
why must you always do a urinalysis in a patient with suspected small vessel vasculitis?
glomerulonephritis can occur
this is asymptomatic until late stage so you actively try to catch it early
what is mononeuritis multiplex?
impairment of blood supply to nerves (usually long nerves eg perineal or radial)
what is the clinical feature when a patient with mononeuritis multiplex of the common perineal nerve can’t extend their ankle?
drop foot
what are ANCAs?
anti-neutrophil cytoplasmic antibodies:
autoantibodies against antigens in the cytoplasm of neutrophil granulocytes
what type of ANCAs do ANCA-associated small vessel vasculitis have?
GPA and EGPA- cANCA
MPA- pANCA
what is localised ANCA-associated vasculitis?
upper/lower resp tract disease
no other systemic involvement or constitutional symptoms
what is early systemic ANCA-associated vasculitis?
any involvement without organ or life threatening involvement
what is generalised ANCA-associated vasculitis?
renal or other organ threatening involvement
what is systemic ANCA-associated vaculitis?
renal or other vital organ failure
what is refractory ANCA-associated vasculitis?
progressive disease unreponsive to steroids + cyclophosphamide
what is the treatment of localised ANCA-associated vasculitis?
methotrexate + steroids
what is the treatment of early systemic ANCA- associated vasculitis?
methotrexate + steroids
what is the treament of generalised ANCA- associated vasculitis?
cyclophosphamide + steroids
rituximab + steroids alternative
what is the treatment of systemic ANCA- associated vasculitis?
cyclophosphamide + steroids
rituximab + steroids alternative
what is the treatment of refractory ANCA-associated vasculitis?
IV immunoglobulins
rituximab
what is Henoch-Schonlein Purpura?
a non-ANCA associated small vessel vasculitis
acute IgA mediated disorder
who tends to get Henoch-Schonlein Purpura?
children 2- 11
but rarely infants
what usually precedes Henoch-Schonlein Purpura?
an upper respiratory infection, pharyngeal infection, GI infection
(a few weeks before)
what is the most common organism involved in the infection preceding Henoch-Schonlein Purpura?
Group A Strep
what is the treatment of Henoch-Schonlein Purpura?
usually self limiting
if evidence of gut or renal involvement- hospital admittance with supportive therapy
what is the difference between purpura and telangiectasia?
purpura doesn’t blanche
telangiectasia does
