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MSK > Vasculitis > Flashcards

Vasculitis Flashcards

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?

A

yes

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
Q

What investigations are done for giant cell arteritis?

A

ESR/CRP - raised

temporal artery biopsy

26
Q

What is the treatment for giant cell arteritis?

A
  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
Q

What investigation result guides treatment in temporal arteritis?

A

falling ESR/CRP

28
Q

What is another name for Wegener’s granulomatosis?

A

granulomatosis with polyangitis

29
Q

What are the histological features of Wegener’s granulomatosis?

A

necrotising, granulomatous vasculitis of arterioles, capillaries and post capillary venules

30
Q

What age groups are affected by Wegener’s?

A

25-60 years old

31
Q

What organ systems does Wegener’s affect?

A

Classical triad:
upper resp tract
lungs
kidneys

other areas are:
skin
nervous system
eye

32
Q

What features of Wegener’s can be seen in the lungs?

A

pulmonary nodules

haemorrhage

33
Q

What features of Wegener’s can be seen in the kidney?

A

glomerulonephritis - haematuria and proteinuria

34
Q

What features of Wegener’s can be seen in the upper resp tract?

A

sinusitis
otitis
nasal crusting/bleeding

35
Q

What features of Wegener’s can be seen in the skin?

A

purpura

ulcers

36
Q

What features of Wegener’s can be seen in the NS?

A

mononeuritis multiplex

CNS vascultitis

37
Q

What features of Wegener’s can be seen in the eye?

A

proptosis
scleritis
episcleritis
uveitis

38
Q

what are the differential diagnoses for pulmonary nodules on CXR?

A

TB
malignancy
S. aureus infection - ie abscess

39
Q

what is the prognosis for untreated GPA?

A

mortality is 90% at 2 years

40
Q

what is the treatment for GPA?

A

Severe:
high dose steroids/cyclophosphamide/biologics

not as severe:
moderate dose steroids
methotrexate
azathioprine

41
Q

What causes diagnostic confusion and delay in vasculitis?

A

highly variable presentation as many organ systems are affected

42
Q

Is giant cell arteritis ANCA positive or negative?

A

ANCA negative - as it is a large vessel disease and ANCA is only present in small/medium vessel disease

MSK (12 decks)

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