Vasculitis Flashcards

(42 cards)

1
Q

what is vasculitis?

A

inflammation and necrosis of blood vessel walls with subsequent impaired flow

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2
Q

What two processes are the result of vasculitis?

A

vessel wall destruction and/or

endothelial injury

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3
Q

What does vessel wall destruction lead to?

A

perforation and haemmorhage

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4
Q

what does endothelial injury result in?

A

thrombosis leading to ischaemia and infarction

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5
Q

what are the histological features of vasculitis?

A

vessel wall infiltration by neutrophils, mononuclear cells and giant cells
fibrinoid necrosis
leukocytoclasis (vascular damage caused by nuclear debris from infiltrating neutrophils)

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6
Q

How is vasculitis classified?

A

size of vessel affected - small, medium, large
target organ(s)
presence or absence of ANCA - anti-neutrophil cytoplasmic antibodies
primary or secondary disease

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7
Q

Name two primary large vessel vasculitis diseases

A
Giant cell (temporal) arteritis 
Takyasu's arteritis
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8
Q

Name two medium/small vessel vasculitic diseases

A

Wegener’s granulomatosis (granulomatosis with polyangitis)
Churg Strauss
microscopic polyangiitis

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9
Q

How does vasculitis present?

A
there is no single presentation:
systemically unwell
fever 
arthralgia 
arthritis
rash
weight loss 
headache 
footdrop
major event eg stroke or bowel infarction
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10
Q

differential diagnoses of vasculitis

A
sepsis
subacute bacterial endocarditis 
hepatitis 
malignancy 
cholesterol emboli
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11
Q

what type of vasculitis is ANCA involved in?

A

small/medium vessel vasculitis

NOT present in large cell vasculitis

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12
Q

how is ANCA detected?

A

indirect immunofluorescence microscopy

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13
Q

what are the two major patterns of ANCA?

A
cytoplasmic ANCA (cANCA)
peri-nuclear ANCA (pAMCA)
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14
Q

What type of vessels does large vessel vasculitis affect?

A

aorta and its branches

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15
Q

Which vessels of the body does temporal arteritis affect?

A

the aorta and larger vessels eg extracranial branches of the carotid arteries

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16
Q

What would be seen on biopsy of the temporal artery in temporal arteritis?

A

granulomas

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17
Q

what is the epidemiology of temporal arteritis?

A

> 50 yrs old
incidence increases with age
twice as common in women

18
Q

How does temporal arteritis present?

A

persistant severe headache
scalp tenderness
jaw claudication
acute blindness - this is a medical emergency
malaise
associated symptoms of polymyalgia rheumatica

19
Q

Are pts with temporal arteritis at risk of stroke?

20
Q

How is temporal arteritis diagnosed?

A

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

21
Q

What would be seen on biopsy of the temporal artery?

A
giant cells 
granulomata
necrotising arteritis 
infiltration of the vessel wall with mononuclear cells 
inflammation 
thickening of the vessel wall
narrowed lumen
22
Q

on examination, what are the qualities seen of the temporal artery?

A

palpable
tender
reduced pulsation

23
Q

What is AION?

A

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).

24
Q

what are the features of the optic disc in AION on fundoscopy?

A

optic disc is pale and swollen

flame shaped haemorrhages at the margin of the optic disc

25
What investigations are done for giant cell arteritis?
ESR/CRP - raised | temporal artery biopsy
26
What is the treatment for giant cell arteritis?
1. 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 2. may need steroid sparing agents to reduce the amount of steroid being taken eg Azathioprine, methotrexate and biologics 3. prophylaxis of osteoporosis - lifestyle advice, calcium/vit D, bisphosphonates, DEXA scan
27
What investigation result guides treatment in temporal arteritis?
falling ESR/CRP
28
What is another name for Wegener's granulomatosis?
granulomatosis with polyangitis
29
What are the histological features of Wegener's granulomatosis?
necrotising, granulomatous vasculitis of arterioles, capillaries and post capillary venules
30
What age groups are affected by Wegener's?
25-60 years old
31
What organ systems does Wegener's affect?
Classical triad: upper resp tract lungs kidneys other areas are: skin nervous system eye
32
What features of Wegener's can be seen in the lungs?
pulmonary nodules | haemorrhage
33
What features of Wegener's can be seen in the kidney?
glomerulonephritis - haematuria and proteinuria
34
What features of Wegener's can be seen in the upper resp tract?
sinusitis otitis nasal crusting/bleeding
35
What features of Wegener's can be seen in the skin?
purpura | ulcers
36
What features of Wegener's can be seen in the NS?
mononeuritis multiplex | CNS vascultitis
37
What features of Wegener's can be seen in the eye?
proptosis scleritis episcleritis uveitis
38
what are the differential diagnoses for pulmonary nodules on CXR?
TB malignancy S. aureus infection - ie abscess
39
what is the prognosis for untreated GPA?
mortality is 90% at 2 years
40
what is the treatment for GPA?
Severe: high dose steroids/cyclophosphamide/biologics not as severe: moderate dose steroids methotrexate azathioprine
41
What causes diagnostic confusion and delay in vasculitis?
highly variable presentation as many organ systems are affected
42
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