Vasculitis Flashcards
What is giant cell arteritis?
large vessel vasculitis
temporal artery
What is GCA linked to?
polymyalgia rheumatic
What is the pathophysiology of GCA?
transmural inflammation
patchy infiltration - multinucleated giant cells
vessel wall thickening –> ichaemia
How does GCA/temporal arteritis present?
scalp tenderness jaw claudication fatigue, malaise, fever temporal headache visual disturbance
How is GCA diagnosed?
increased inflammatory markers
temporal artery biopsy
Why is a temporal biopsy sometimes negative in GCA?
patchy involvement
How is GCA managed?
prednisolone 40mg (60mg if visual symptoms)
treatment should not be delayed while waiting for biopsy
What is takayasu arteritis?
large vessel vasculitis
aorta
What does occlusion of the aorta cause in takayasu arteritis?
absent limb pulse
upper and lower limb claudication
How does takayasu arteritis present?
malaise, headache
unequal blood pressure in upper limbs
upper and lower limb claudication
How is takayasu arteritis managed?
prednisolone
Who typically gets Kawasaki disease?
Children
Who typically gets GCA?
over 50s
What is Kawasaki disease?
medium vessel vasculitis
How does Kawasaki disease present?
high grade fever conjunctival injection bright red, dry, cracked lips strawberry tongue red palms and soles
How is Kawasaki disease diagnosed and managed?
clinical diagnosis
aspirin
IV immunoglobulin
What is a complication of Kawasaki disease?
coronary artery aneurysm
What is polyarteritis nodosa associated with?
hep B infection
What is polyarteritis nodosa?
necrotising inflammation leading to aneurysm formation
What is granulomatosis with polyangitis?
Wegener’s
ANCA associated small vessel vasculitis
What is the pathophysiology of GPA?
granulomatous inflammation (involving resp tract) + necrotising vasculitis of small vessels
How does granulomatosis with polyangitis present?
ENT symptoms (common)
- nose bleeds
- nasal crusting and congestion
resp symptoms
- haemoptysis
palpable purpuric rash
fever, weight loss, night sweats
necrotising glomerulonephritis
How is GPA diagnosed?
c-ANCA
FBC - anaemia
CXR - cavitating lesions
renal biopsy - epithelial crescents in Bowman’s capsule
How is GPA managed?
steroids
cyclophosphamide
What is eosinophilic GPA?
Churg Strauss
ANCA associated small vessel vasculitis
What is the pathophysiology of eGPA?
eosinophilic granulomatous inflammation and necrotising vasculitis of small vessels
How does eGPA present?
late onset asthma
rhinitis
high eosinophil count
similar to GPA
How is eGPA diagnosed?
pANCA
same as GPA
How is eGPA managed?
IV steroids
cyclophosphamide
What is microscopic polyangitis?
ANCA associated small vessel vasculitis (pANCA)
How does microscopic polyangitis present?
systemic symptoms + kidney failure
What is Henoch Schonlein purpura?
IgA mediated small vessel vasculitis
What is Henoch Shonlein associated with?
IgA nephropathy
How does Henoch Schonlein present?
joint pain
abdo pain
purpuric rash over butocks and lower limb
How is Henoch Schonlein managed?
supportive - self limiting
analgesia
