Systemic inflammatory vasculitis

Question 1

what is vasculitis

Answer 1

inflammation of the blood vessel walls that can be seen in many diseases including SLE, RA, polymyositis & some allergic drug reactions

Question 2

common features of the the systemic vasculitides

Answer 2

anaemia and raised ESR

All rare except for giant cell (temporal) arteritis

Question 3

stages of vasculitis

Answer 3

• inflammation and necrosis of blood vessel walls - impaired blood flow
• perforation and haemorrhage into tissues
• endothelial injury = thrombosis and ischaemia / infarction of dependent tissues

Question 4

vasculitides affecting large vessels e.g. aorta and its major branches

Answer 4

1. giant cell arteritis / polymyalgia rheumatica

2. takayasu’s arteritis

Question 5

vasculitides affecting medium vessels (main visceral vessels e.g. renal, coronary)

Answer 5

1. polyarteritis nodosa

2. kawasaki’s disease (affects children

Question 6

vasculitides affecting small vessels (small arteries, arterioles, venules and capillaries)

Answer 6

1. ANCA positive - microscopic polyangitis, Wegener’s granulomatosis, Churg-Straus syndrome
2. ANCA negative - Henoch-Schonlein purpura, cutaneous leucocytoclastic vasculities, essential cryoglobulinaemia

Question 7

ANCA stands for…

Answer 7

antineutrophil cytoplasmic antibodies

Question 8

giant cell arteritis / polymyalgia rheumatica clinical features

Answer 8

• PMR = stiffness and intense pain in muscles of neck, shoulder, hips and lumbar spine
• Constitutional symps
• Inflammation of superficial temporal arteries = headache, tenderness over scalp/temple. Can cause stroke / sudden loss of vision

Question 9

giant cell arteritis / polymyalgia rheumatica investigations

Answer 9

• rx started on clin diag
• high ESR and CRP
• normochromic normocytic anaemia
• temporal artery biopsy performed in GCA suspected before starting steroids

Question 10

management giant cell arteritis / polymyalgia rheumatica

Answer 10

• corticosteroids: prednisolone, dose gradually reduced

- prophylaxis against steroid induced osteoporosis

Question 11

takayasu’s arteritis

Answer 11

rare except in japan
vasculitis involving aortic arch and other major arteries causes HTN, absent peripheral pulses, stroke and cardiac failure
rx - corticosteroids

Question 12

polyarteritis nodosa affects predom…

Answer 12

middle aged men

Question 13

polyarteritis nodosa is secondary to

Answer 13

deposition of immune complexes

Question 14

polyarteritis nodosa clinical features

Answer 14

fever, malaise, weight loss, mononeuritis multiplex, abdo pain, renal impairment and HTN

Question 15

polyarteritis nodosa diagnosis

Answer 15

angiography - visualise microaneurysms

biopsy of affected organ, often the kidney

Question 16

polyarteritis nodosa rx

Answer 16

corticosteroids usually in combination with immunosuppressive drugs (azathioprine, cyclophosphamide)

Question 17

microscopic polyarteritis (polyangiitis) what is it

Answer 17

involves lungs and kidney = haemoptysis, haematuria, proteinuria & progressive renal failure

Question 18

diagnosis microscopic polyarteritis (polyangiitis)

Answer 18

renal biopsy

measurement of serum perinuclear (p)-ANCA, present in 60%

Question 19

rx microscopic polyarteritis (polyangiitis)

Answer 19

corticosteroids usually in combination with immunosuppressive drugs (azathioprine, cyclophosphamide)

Question 20

churg-strauss syndrome

Answer 20

rare

triad of asthma, eosinophilia, systemic vasculitis affecting the nerves and skin

Question 21

rx churg-strauss syndrome

Answer 21

corticosteroids usually in combination with immunosuppressive drugs (azathioprine, cyclophosphamide)

Question 22

henoch-schonlein purpura presents w?

Answer 22

kids
purpuric rash on legs and buttocks
abdo pain, arthritis, haematuria, nephritis

Question 23

henoch-schonlein purpura characterised by

Answer 23

vascular deposition of IgA-dominant immune complexes, and the onset is often preceded by an acute upper resp tract infection

Question 24

henoch-schonlein purpura rx

Answer 24

recovery usually spontaneous

Question 25

behcets disease

Answer 25

rare
recurrent oral ulceration
rx w immunosuppressive therapy (steroids, ciclosporin)