Vasculitis

Question 1

What are the different categories of vasculitis?

Answer 1

Large vessel
Medium vessel
Small vessel:
- ANCA associated
- Immune complex

Question 2

What are the large vessel vasculitides?

Answer 2

Takayasu arteritis

Giant cell/temporal arteritis

Question 3

What are the medium vessel vasculitides?

Answer 3

Polyarteritis nodosa

Kawasaki disease

Question 4

What are the ANCA-associated small vessel vasculitides?

Answer 4

Microscopic polyangitis
Granulomatosis with polyangitis
Eosinophilic granulomatosis with polyangitis

Question 5

What are the immune complex small vessel vasculitides?

Answer 5

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

Question 6

What is the pathology of large vessel vasculitis?

Answer 6

Chronic granulomatous inflammation of aorta + major branches

Question 7

Who gets Takayasu arteritis?

Answer 7

Women, < 40

More common in Asian population

Question 8

Who gets GCA?

Answer 8

> 50 years old

Question 9

What are the clinical features of GCA?

Answer 9

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

Question 10

What causes jaw claudication in GCA?

Answer 10

Ischaemia of maxillary artery

Question 11

What causes the visual loss in GCA?

Answer 11

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

Question 12

What might you see on examination of someone with GCA?

Answer 12

Tender, enlarged, non-pulsatile temporal arteries

Question 13

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

Answer 13

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

Question 14

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

Answer 14

Temporal artery USS

Question 15

What is the treatment for GCA?

Answer 15

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

Question 16

Which constitutional symptoms are often seen with large vessel vasculitides?

Answer 16

Fever
Malaise
Night sweats
Weight loss
Arthralgia
Fatigue

Question 17

What are the clinical features of Takayasu arteritis?

Answer 17

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

Question 18

Which investigations should be done to diagnose TA?

Answer 18

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

Question 19

How is TA treated?

Answer 19

40-60mg prednisolone

+/- methotrexate or azathioprine

Question 20

What are the common features of small vessel vasculitides?

Answer 20

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

Question 21

What is the old name for GPA?

Answer 21

Wegener’s granulomatosis

Question 22

What are the clinical features of GPA?

Answer 22

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

Question 23

Which blood markers are associated with GPA?

Answer 23

cANCA + PR3

Question 24

What is the old name for EGPA?

Answer 24

Churg-Strauss syndrome

Question 25

What are the clinical features of EGPA?

Answer 25

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

Question 26

Which blood markers are associated with EGPA?

Answer 26

pANCA

Question 27

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

Answer 27

Similar to other small vessel vasculitis | Glomerulonephritis very common (in up to 90%)

Question 28

Which blood markers are associated with MPA?

Answer 28

pANCA + MPO

Question 29

What is the management of ANCA associated vasculitis?

Answer 29

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

Question 30

What is Henoch-Schonlein Purpura?

Answer 30

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

Question 31

Who gets HSP?

Answer 31

Children age 2-11

Question 32

What is the classic presentation of HSP?

Answer 32

URTI (group A strep), 1-3 weeks later --> - purpuric rash on buttocks/legs - colicky abdominal pain - bloody diarrhoea - joint pain/swelling - glomerulonephritis in 50%

Question 33

Which investigations should be done in HSP?

Answer 33

ESSENTIAL to do urinalysis to check for renal involvement

Question 34

What is the management + prognosis of HSP?

Answer 34

Symptomatic - usually self-limiting, resolves within 8 weeks