Vasculitis Flashcards

1
Q

What is giant cell arteritis?

A

large vessel vasculitis

temporal artery

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2
Q

What is GCA linked to?

A

polymyalgia rheumatic

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3
Q

What is the pathophysiology of GCA?

A

transmural inflammation
patchy infiltration - multinucleated giant cells

vessel wall thickening –> ichaemia

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4
Q

How does GCA/temporal arteritis present?

A
scalp tenderness 
jaw claudication 
fatigue, malaise, fever
temporal headache 
visual disturbance
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5
Q

How is GCA diagnosed?

A

increased inflammatory markers

temporal artery biopsy

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6
Q

Why is a temporal biopsy sometimes negative in GCA?

A

patchy involvement

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7
Q

How is GCA managed?

A

prednisolone 40mg (60mg if visual symptoms)

treatment should not be delayed while waiting for biopsy

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8
Q

What is takayasu arteritis?

A

large vessel vasculitis

aorta

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9
Q

What does occlusion of the aorta cause in takayasu arteritis?

A

absent limb pulse

upper and lower limb claudication

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10
Q

How does takayasu arteritis present?

A

malaise, headache
unequal blood pressure in upper limbs
upper and lower limb claudication

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11
Q

How is takayasu arteritis managed?

A

prednisolone

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12
Q

Who typically gets Kawasaki disease?

A

Children

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13
Q

Who typically gets GCA?

A

over 50s

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14
Q

What is Kawasaki disease?

A

medium vessel vasculitis

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15
Q

How does Kawasaki disease present?

A
high grade fever 
conjunctival injection 
bright red, dry, cracked lips 
strawberry tongue 
red palms and soles
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16
Q

How is Kawasaki disease diagnosed and managed?

A

clinical diagnosis

aspirin
IV immunoglobulin

17
Q

What is a complication of Kawasaki disease?

A

coronary artery aneurysm

18
Q

What is polyarteritis nodosa associated with?

A

hep B infection

19
Q

What is polyarteritis nodosa?

A

necrotising inflammation leading to aneurysm formation

20
Q

What is granulomatosis with polyangitis?

A

Wegener’s

ANCA associated small vessel vasculitis

21
Q

What is the pathophysiology of GPA?

A

granulomatous inflammation (involving resp tract) + necrotising vasculitis of small vessels

22
Q

How does granulomatosis with polyangitis present?

A

ENT symptoms (common)

  • nose bleeds
  • nasal crusting and congestion

resp symptoms
- haemoptysis

palpable purpuric rash

fever, weight loss, night sweats

necrotising glomerulonephritis

23
Q

How is GPA diagnosed?

A

c-ANCA
FBC - anaemia
CXR - cavitating lesions
renal biopsy - epithelial crescents in Bowman’s capsule

24
Q

How is GPA managed?

A

steroids

cyclophosphamide

25
Q

What is eosinophilic GPA?

A

Churg Strauss

ANCA associated small vessel vasculitis

26
Q

What is the pathophysiology of eGPA?

A

eosinophilic granulomatous inflammation and necrotising vasculitis of small vessels

27
Q

How does eGPA present?

A

late onset asthma
rhinitis
high eosinophil count
similar to GPA

28
Q

How is eGPA diagnosed?

A

pANCA

same as GPA

29
Q

How is eGPA managed?

A

IV steroids

cyclophosphamide

30
Q

What is microscopic polyangitis?

A

ANCA associated small vessel vasculitis (pANCA)

31
Q

How does microscopic polyangitis present?

A

systemic symptoms + kidney failure

32
Q

What is Henoch Schonlein purpura?

A

IgA mediated small vessel vasculitis

33
Q

What is Henoch Shonlein associated with?

A

IgA nephropathy

34
Q

How does Henoch Schonlein present?

A

joint pain
abdo pain
purpuric rash over butocks and lower limb

35
Q

How is Henoch Schonlein managed?

A

supportive - self limiting

analgesia