Vasculitis Flashcards
what is vasculitis?
inflammation and necrosis of blood vessel walls with subsequent impaired flow
What two processes are the result of vasculitis?
vessel wall destruction and/or
endothelial injury
What does vessel wall destruction lead to?
perforation and haemmorhage
what does endothelial injury result in?
thrombosis leading to ischaemia and infarction
what are the histological features of vasculitis?
vessel wall infiltration by neutrophils, mononuclear cells and giant cells
fibrinoid necrosis
leukocytoclasis (vascular damage caused by nuclear debris from infiltrating neutrophils)
How is vasculitis classified?
size of vessel affected - small, medium, large
target organ(s)
presence or absence of ANCA - anti-neutrophil cytoplasmic antibodies
primary or secondary disease
Name two primary large vessel vasculitis diseases
Giant cell (temporal) arteritis Takyasu's arteritis
Name two medium/small vessel vasculitic diseases
Wegener’s granulomatosis (granulomatosis with polyangitis)
Churg Strauss
microscopic polyangiitis
How does vasculitis present?
there is no single presentation: systemically unwell fever arthralgia arthritis rash weight loss headache footdrop major event eg stroke or bowel infarction
differential diagnoses of vasculitis
sepsis subacute bacterial endocarditis hepatitis malignancy cholesterol emboli
what type of vasculitis is ANCA involved in?
small/medium vessel vasculitis
NOT present in large cell vasculitis
how is ANCA detected?
indirect immunofluorescence microscopy
what are the two major patterns of ANCA?
cytoplasmic ANCA (cANCA) peri-nuclear ANCA (pAMCA)
What type of vessels does large vessel vasculitis affect?
aorta and its branches
Which vessels of the body does temporal arteritis affect?
the aorta and larger vessels eg extracranial branches of the carotid arteries
What would be seen on biopsy of the temporal artery in temporal arteritis?
granulomas
what is the epidemiology of temporal arteritis?
> 50 yrs old
incidence increases with age
twice as common in women
How does temporal arteritis present?
persistant severe headache
scalp tenderness
jaw claudication
acute blindness - this is a medical emergency
malaise
associated symptoms of polymyalgia rheumatica
Are pts with temporal arteritis at risk of stroke?
yes
How is temporal arteritis diagnosed?
3 or more of:
>50 yrs
new headache
temporal artery tenderness or decreased pulsation
ESR >50
abnormal temporal artery biopsies showing necrotising arteritis with mononuclear infiltrate or granulomatous inflammation
What would be seen on biopsy of the temporal artery?
giant cells granulomata necrotising arteritis infiltration of the vessel wall with mononuclear cells inflammation thickening of the vessel wall narrowed lumen
on examination, what are the qualities seen of the temporal artery?
palpable
tender
reduced pulsation
What is AION?
occurs in giant cell arteritis
anterior/arteritic ischaemic optic neuropathy
sudden, painless, monocular and severe visual loss
may be preceded by transient visual loss
may describe a curtain coming down over one eye
medical condition involving loss of vision caused by damage to the optic nerve as a result of insufficient blood supply (ischemia).
what are the features of the optic disc in AION on fundoscopy?
optic disc is pale and swollen
flame shaped haemorrhages at the margin of the optic disc
What investigations are done for giant cell arteritis?
ESR/CRP - raised
temporal artery biopsy
What is the treatment for giant cell arteritis?
- prompt corticosteroids ie Prednisolone, may be IV, dose depends on whether there is visual disturbance or not - higher dose for visual disturbance, take prednisolone for 1-2 years
- may need steroid sparing agents to reduce the amount of steroid being taken eg Azathioprine, methotrexate and biologics
- prophylaxis of osteoporosis - lifestyle advice, calcium/vit D, bisphosphonates, DEXA scan
What investigation result guides treatment in temporal arteritis?
falling ESR/CRP
What is another name for Wegener’s granulomatosis?
granulomatosis with polyangitis
What are the histological features of Wegener’s granulomatosis?
necrotising, granulomatous vasculitis of arterioles, capillaries and post capillary venules
What age groups are affected by Wegener’s?
25-60 years old
What organ systems does Wegener’s affect?
Classical triad:
upper resp tract
lungs
kidneys
other areas are:
skin
nervous system
eye
What features of Wegener’s can be seen in the lungs?
pulmonary nodules
haemorrhage
What features of Wegener’s can be seen in the kidney?
glomerulonephritis - haematuria and proteinuria
What features of Wegener’s can be seen in the upper resp tract?
sinusitis
otitis
nasal crusting/bleeding
What features of Wegener’s can be seen in the skin?
purpura
ulcers
What features of Wegener’s can be seen in the NS?
mononeuritis multiplex
CNS vascultitis
What features of Wegener’s can be seen in the eye?
proptosis
scleritis
episcleritis
uveitis
what are the differential diagnoses for pulmonary nodules on CXR?
TB
malignancy
S. aureus infection - ie abscess
what is the prognosis for untreated GPA?
mortality is 90% at 2 years
what is the treatment for GPA?
Severe:
high dose steroids/cyclophosphamide/biologics
not as severe:
moderate dose steroids
methotrexate
azathioprine
What causes diagnostic confusion and delay in vasculitis?
highly variable presentation as many organ systems are affected
Is giant cell arteritis ANCA positive or negative?
ANCA negative - as it is a large vessel disease and ANCA is only present in small/medium vessel disease
