Vasculitis Flashcards
vasculitis
inflammation of blood vessels often with ischaemia, necrosis & organ inflammation
primary vasculitis
results from an inflammatory response that targets the vessel walls and has no known cause. Sometimes this is autoimmune
secondary vasculitis
may be triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer
what are the 2 main causes of large vessel vasculitis?
takayasu arteritis (TA) giant cell arteritis (GCA)
who does TA predominantly affect?
under 40 years
females
Asians
who does GCA usually affect?
over 50 years
what does GCA usually cause?
temporal arteritis
what is large vessel vasculitis characterised by?
granulomatous infiltration of the walls of the large vessels
what are the common clinical findings of large vessel vasculitis?
bruit (of the carotid artery) BP difference of extremitis claudication carotodynia/vessel tenderness hypertension
carotodynia
tenderness of the carotid artery
what are the classic symptoms of temporal areteritis?
unilateral temporal headache
scalp tenderness
jaw claudication
what is there a risk of in temporal arteritis & why?
blindness due to ischaemia of the optic nerve
what medium vessel vasculitis is seen in children usually
kawasaki disease
what are the most important vessels that kawasaki disease can affect?
coronary arteries
what is polyarteritis nodosa characterised by?
necrotizing inflammatory lesions that affect arteries at vessel bifurcations
what does polyarteritis nodosa result in?
microaneurysm formation and aneurysms
what is polyarteritis nodosa associated with?
Hep. B
what organs can polyarteritis nodosa affect?
skin
gut
kidneys
Wegner’s granulomatosis
granulomatosis with polyangitis (GPA)
what is the pathology of GPA?
Granulomatous inflammation of respiratory tract, small and medium vessels. Necrotising
Churg-Strauss Syndrome
eosinophilic granulomatosis with polyangitis (EGPA)
what is the pathology of EGPA?
Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels. Associated with asthma
what is the pathology of MPA?
Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis very common
MPA
microscopic polyangitis
what are the 3 types of ANCA associated small vessel vasculitis?
GPA
EGPA
MPA
what are the 2 main types of non-ANCA associated small vessel vasculitis?
Henoch Schnlein purpura
cryoglobulinaemia
which sex is GPA more common in?
men
what age does GPA typically present at?
33-55 years
what other systems can GPA affect?
ENT Resp Cutaneous Renal CNS Ocular
what is the main clinical difference between EGPA & GPA?
present of late onset asthma & high eosiniophil count in EGPA
ANCA
anti-neutrophil cytoplasmic antibodies
anti-neutrophil cytpolasmic antibodies?
a group of autoantibodies against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell)
what is used to detect ANCA & can differentiate ANCA patterns?
immunofluorescence
Henoch-Schonlein Purpura (HSP)
an acute immunoglobulin A (IgA)–mediated disorder