Large vessel vasculitis

Question 1

What normally causes a vasculitis

Answer 1

Usually an autoimmune phenomena. Can rarely be triggered by direct inoculation of bacteria e.g. fragments from bacterial endocarditis.

Question 2

What do we mean by large vessels?

Answer 2

aorta

branches off the aorta e.g. subclavian

Question 3

what is the most common cause of vasculitis in the uk?

Answer 3

Henoch-schonlein purpura. An immune-complex mediated vasculitis (involves IgA antibodies directly targeting own endothelial cells). The immune complexes get produced after an URTI or a strep throat. Immune complexes deposited in SMALL blood vessels. Generally self limiting. Can give steroids in severe cases.

Question 4

what is the most common type of vasculitis in ADULTS

Answer 4

Giant cell arteritis

Question 5

what age group does GCA tend to affect?

Answer 5

classically only those over the age of 50

Question 6

what is takayasu’s vasculitis?

Answer 6

rare condition. common in Japan. affects young women. It is defined as “granulomatous inflammation of the aorta and its major branches”

Question 7

What are the CRANIAL SYMPTOMS of GCA?

Answer 7

• headache - new onset, severe, doesn’t go away with analgesia
• scalp tenderness
• neck ache (potentially due to involvement of vertebral arteries)
• jaw claudication
• polymagia rheumatica - shoulder and hip girdle stiffness (worse in morning and eases up through day)
• visual symptoms

Question 8

What are the SYSTEMIC SYMPTOMS of GCA?

Answer 8

• Constitutional
• weight loss
• fever
• limb claudication
• limb claudication (subclavian artery involvement)
• abdo pain (mesenteric artery involvement)
• hypertension

Question 9

Clinical signs of GCA

Answer 9

inflamed temporal artery

Question 10

what to do in suspected GCA

Answer 10

give 60mg prednisolone

refer to ophthalmology immediately if visual symptoms

Question 11

What are the ophthalmic complications of GCA?

Answer 11

arteritic anterior ischaemic neuropathy (present with amaurosis fugax or complete vision loss)
central retinal artery occlusion (cherry red spot)
slow flow retinopathy (pixelations)
cilioretinal artery occlusion
cranial nerve palsies (diplopia

Question 12

temporal artery USS shows what in GCA?

Answer 12

halo sign

Question 13

How do we confirm temporal arteritis?

Answer 13

biopsy. Hall mark is giant cells on histology (multi-nucleated cells)

Question 14

GCA diagnostic criteria

Answer 14

3 out of 5:

• age >50
• new headache
• temporal artery anomaly (tenderness/decreased pulsation)
• ESR >50
• Abnormal artery biopsy

Question 15

what are the complication of steroid therapy in GCA

Answer 15

osteoporosis + fracture
poor diabetes control
cushings
infection

Question 16

GCA can present with no headache and may be an incidental finding when investigating what conditions?

Answer 16

anaemia
weight loss
raised inflammatory markers
arm pain/colour change/claudicant symptoms
PUO
thoracic aneurisms from asymptomatic aortitis
posterior circulation stroke
DO A PET-CT SCAN

Question 17

what is takayasus arteritis

Answer 17

a granulomatous pan-arteritis affecting the aorta and its branches. It has 2 main phases: an inflammatory phase when the person has feverish constitutional symptoms, and then an ischaemic pulseless phase which is considered to be “burnt out”. We ideally want to pick people up in the inflammatory phase

Question 18

how does takayasus arteritis present?

Answer 18

arthralgia, myalgia, chest wall pain
fever, weight loss, visual symptoms
upper limb claudication
hypertension, new murmur (aortic root dilatation),
carotidynia (pain in the neck)
coronary artery involvement
UNEXPLAINED ACUTE PHASE RESPONSE
PUO
FEMALE PREDOMINANT DISEASE

Question 19

How is takayasus arteritis treated?

Answer 19

steroids, methotrexate, cyclophosphamide
tocilizumab (IL-6 blocker)
manage cardio risk factors
consider endovascular surgery as a last resort in inactive disease only