VASCULITIS

Question 1

What happens in the vessel during vasculitis?

Answer 1

Dendritic cells –> cytokine release –> T cell activation –> Macrophage activation –> Progressive inflammatory response, endothelial damage –> Intima hyperplasia

Question 2

What can be some systemic symptoms of vasculitis?

Answer 2

Fever, malaise, weight loss and fatigue

Question 3

What are the two large vessel vasculitis?

Answer 3

Giant cell arteritis (Over 50, temporal), Takayasu arteritis (Under 40, females, Asains)

Question 4

What are the presenting features of large vessel vasculitis?

Answer 4

Bruit, blood pressure difference of extremities, claudication, Carotodynia or vessel tenderness, hypertension

Question 5

What condition is polymyalgia rheumatica associated with?

Answer 5

Temporal arteritis - GCA

Question 6

What are the presenting symptoms?

Answer 6

Scalp tenderness, unilateral temporal headache, jaw claudication - risk of blindness, ischaemia of the optic nerve

Question 7

Is there a specific blood test for temporal arteritis?

Answer 7

Nope, there is no specific antibody involvement

Question 8

What age group is affected by Kawasaki disease? Where does it affect?

Answer 8

Under 5s

Can affect any vessels but affects the coronary arteries - can cause aneurysm

Question 9

What can polyarteritis nodosa cause?

Answer 9

Lesions at vessel bifurcations, micro aneurysm formation

Question 10

What are medium vessel vasculitis associated with?

Answer 10

Hep B

Question 11

What is the new name for Wegener’s syndrome?

Answer 11

Granulomatosis with polyangitis - Granulomatous inflammation of respiratory tract, small and medium vessels. Necrotising glomerulonephritis common.

Question 12

What is the new name for Churg Strauss syndrome?

Answer 12

Eosinophilic granulomatosis with polyangiitis (EGPA)

- Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels. Associated with asthma

Question 13

What is the new name for Microscopic polyangiitis?

Answer 13

Trick question! Its not changed!
Affects kidneys, not so much lungs – doesn’t have the multisystem presentation
Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis very common

Question 14

What are the symptoms and signs of granulomatosis with polyangitis?

Answer 14

• Nasal or oral inflammation, Chest nodules or cavities (cough, haemoptysis), microhematuria
• Sinusitis, nasal crusting, epistaxis, sensorineural deafness, otitis media, Saddle nose (cartilage ischaemia), palpable purpura (non blanching cutaneous lesions)
• Necrotising glomerulonephritis – blood and protein in urine
• Mononeuritis multiplex, cranial nerve palsy (would usually cause double vision)
• Conjunctivitis, uveitis

Question 15

What is the main difference between GPA and EGPA?

Answer 15

The late onset of asthma and the high eosinophil count

Question 16

What does Anti PR3 go along with?

Answer 16

cANCA - GPA

Question 17

What does Anti MPO go along with?

Answer 17

pANCA - MPA and EGPA

Question 18

How would you manage localised/ early systemic AAV?

Answer 18

Methotrexate and steroids

Question 19

How would you manage generalised systemic AAV?

Answer 19

Cyclophosphamide and steroids

Question 20

What may be a risk factor of AAV?

Answer 20

Nasal carriage of S.aureus

Question 21

How would you describe Henoch - Schonlein Purpura

Answer 21

Acute generalised IgA mediated disorder - generalised vasculitis

Question 22

What is Henoch - Schonlein purpura presentation?

Answer 22

• Purpuric rash over bum and lower limbs, colicky abdominal pain, bloody diarrhoea, joint pain and swelling and renal involvement