Vasculitis Flashcards

1
Q

What are the different categories of vasculitis?

A
Large vessel
Medium vessel
Small vessel:
- ANCA associated
- Immune complex
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2
Q

What are the large vessel vasculitides?

A

Takayasu arteritis

Giant cell/temporal arteritis

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3
Q

What are the medium vessel vasculitides?

A

Polyarteritis nodosa

Kawasaki disease

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4
Q

What are the ANCA-associated small vessel vasculitides?

A

Microscopic polyangitis
Granulomatosis with polyangitis
Eosinophilic granulomatosis with polyangitis

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5
Q

What are the immune complex small vessel vasculitides?

A

Cryoglobulinaemic vasculitis
IgA vasculitis (Henoch-Schonlein)
Hypocomplementemic urticarial vasculitis

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6
Q

What is the pathology of large vessel vasculitis?

A

Chronic granulomatous inflammation of aorta + major branches

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7
Q

Who gets Takayasu arteritis?

A

Women, < 40

More common in Asian population

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8
Q

Who gets GCA?

A

> 50 years old

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9
Q

What are the clinical features of GCA?

A

Headache - continuous, temporal/occipital
Scalp tenderness e.g. hair combing
Jaw claudication - fatigue/discomfort on chewing/speaking (pathognomonic of GCA)
Visual loss

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10
Q

What causes jaw claudication in GCA?

A

Ischaemia of maxillary artery

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11
Q

What causes the visual loss in GCA?

A

Amaurosis fugax - loss of blood supply to eye
-> swollen optic disc
Permanent in about 20%

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12
Q

What might you see on examination of someone with GCA?

A

Tender, enlarged, non-pulsatile temporal arteries

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13
Q

What is the gold standard for diagnosis of GCA and what would it show?

A

Temporal artery biopsy –> asap
- 100% specificity but only 15-40% sensitivity due to patchy involvement of artery

Mononuclear infiltration or granulomatous inflammation, usually with multinucleate giant cells

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14
Q

Which investigation might be helpful if biopsy negative but clinical suspicion of GCA?

A

Temporal artery USS

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15
Q

What is the treatment for GCA?

A

Rapid corticosteroids to avoid loss of vision –> do not delay to wait for biopsy
- prednisolone 40mg if no vision impairment
- 60mg if vision affected
Reduce dose gradually over about 2 years

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16
Q

Which constitutional symptoms are often seen with large vessel vasculitides?

A
Fever
Malaise
Night sweats
Weight loss
Arthralgia
Fatigue
17
Q

What are the clinical features of Takayasu arteritis?

A

Claudication symptoms in both upper and lower limbs
Reduced pulses
Difference in BP between limbs
Artery bruits

18
Q

Which investigations should be done to diagnose TA?

A

Raised inflammatory markers
BP
MR angiogram –> thick vessel walls + stenosis

19
Q

How is TA treated?

A

40-60mg prednisolone

+/- methotrexate or azathioprine

20
Q

What are the common features of small vessel vasculitides?

A
Fever + weight loss
Raised, non-blanching purpuric rash
Arthralgia/arthritis
Mononeuritis multiplex
Glomerulonephritis
Lung opacities on CXR
21
Q

What is the old name for GPA?

A

Wegener’s granulomatosis

22
Q

What are the clinical features of GPA?

A
Common features of small vessel vasculitis +
ENT symptoms: 
- nose bleeds 
- deafness
- recurrent sinusitis
- nasal crusting
- 'saddle nose' (collapsing of cartilage)
Haemoptysis + caveatting lesions on CXR
23
Q

Which blood markers are associated with GPA?

A

cANCA + PR3

24
Q

What is the old name for EGPA?

A

Churg-Strauss syndrome

25
Q

What are the clinical features of EGPA?

A
Late onset asthma
Rhinitis
High peripheral blood eosinophil count
Mononeuritis multiplex common
Other features similar to GPA
26
Q

Which blood markers are associated with EGPA?

A

pANCA

27
Q

What are the clinical features of microscopic polyangiitis (MPA)?

A

Similar to other small vessel vasculitis

Glomerulonephritis very common (in up to 90%)

28
Q

Which blood markers are associated with MPA?

A

pANCA + MPO

29
Q

What is the management of ANCA associated vasculitis?

A
Localised/early systemic:
--> methotrexate + steroids
Generalised/systemic (most patients):
--> IV steroids + cyclophosphamide
Refractory:
--> IV Ig + rituximab
30
Q

What is Henoch-Schonlein Purpura?

A

Acute IgA mediated vasculitis primarily affecting GI tract, kidneys + joints

31
Q

Who gets HSP?

A

Children age 2-11

32
Q

What is the classic presentation of HSP?

A

URTI (group A strep), 1-3 weeks later –>

  • purpuric rash on buttocks/legs
  • colicky abdominal pain
  • bloody diarrhoea
  • joint pain/swelling
  • glomerulonephritis in 50%
33
Q

Which investigations should be done in HSP?

A

ESSENTIAL to do urinalysis to check for renal involvement

34
Q

What is the management + prognosis of HSP?

A

Symptomatic - usually self-limiting, resolves within 8 weeks