Vasculitis

Question 1

What is giant cell arteritis?

Answer 1

large vessel vasculitis

temporal artery

Question 2

What is GCA linked to?

Answer 2

polymyalgia rheumatic

Question 3

What is the pathophysiology of GCA?

Answer 3

transmural inflammation
patchy infiltration - multinucleated giant cells

vessel wall thickening –> ichaemia

Question 4

How does GCA/temporal arteritis present?

Answer 4

scalp tenderness 
jaw claudication 
fatigue, malaise, fever
temporal headache 
visual disturbance

Question 5

How is GCA diagnosed?

Answer 5

increased inflammatory markers

temporal artery biopsy

Question 6

Why is a temporal biopsy sometimes negative in GCA?

Answer 6

patchy involvement

Question 7

How is GCA managed?

Answer 7

prednisolone 40mg (60mg if visual symptoms)

treatment should not be delayed while waiting for biopsy

Question 8

What is takayasu arteritis?

Answer 8

large vessel vasculitis

aorta

Question 9

What does occlusion of the aorta cause in takayasu arteritis?

Answer 9

absent limb pulse

upper and lower limb claudication

Question 10

How does takayasu arteritis present?

Answer 10

malaise, headache
unequal blood pressure in upper limbs
upper and lower limb claudication

Question 11

How is takayasu arteritis managed?

Answer 11

prednisolone

Question 12

Who typically gets Kawasaki disease?

Answer 12

Children

Question 13

Who typically gets GCA?

Answer 13

over 50s

Question 14

What is Kawasaki disease?

Answer 14

medium vessel vasculitis

Question 15

How does Kawasaki disease present?

Answer 15

high grade fever 
conjunctival injection 
bright red, dry, cracked lips 
strawberry tongue 
red palms and soles

Question 16

How is Kawasaki disease diagnosed and managed?

Answer 16

clinical diagnosis

aspirin
IV immunoglobulin

Question 17

What is a complication of Kawasaki disease?

Answer 17

coronary artery aneurysm

Question 18

What is polyarteritis nodosa associated with?

Answer 18

hep B infection

Question 19

What is polyarteritis nodosa?

Answer 19

necrotising inflammation leading to aneurysm formation

Question 20

What is granulomatosis with polyangitis?

Answer 20

Wegener’s

ANCA associated small vessel vasculitis

Question 21

What is the pathophysiology of GPA?

Answer 21

granulomatous inflammation (involving resp tract) + necrotising vasculitis of small vessels

Question 22

How does granulomatosis with polyangitis present?

Answer 22

ENT symptoms (common)

• nose bleeds
• nasal crusting and congestion

resp symptoms
- haemoptysis

palpable purpuric rash

fever, weight loss, night sweats

necrotising glomerulonephritis

Question 23

How is GPA diagnosed?

Answer 23

c-ANCA
FBC - anaemia
CXR - cavitating lesions
renal biopsy - epithelial crescents in Bowman’s capsule

Question 24

How is GPA managed?

Answer 24

steroids

cyclophosphamide

Question 25

What is eosinophilic GPA?

Answer 25

Churg Strauss | ANCA associated small vessel vasculitis

Question 26

What is the pathophysiology of eGPA?

Answer 26

eosinophilic granulomatous inflammation and necrotising vasculitis of small vessels

Question 27

How does eGPA present?

Answer 27

late onset asthma rhinitis high eosinophil count similar to GPA

Question 28

How is eGPA diagnosed?

Answer 28

pANCA | same as GPA

Question 29

How is eGPA managed?

Answer 29

IV steroids | cyclophosphamide

Question 30

What is microscopic polyangitis?

Answer 30

ANCA associated small vessel vasculitis (pANCA)

Question 31

How does microscopic polyangitis present?

Answer 31

systemic symptoms + kidney failure

Question 32

What is Henoch Schonlein purpura?

Answer 32

IgA mediated small vessel vasculitis

Question 33

What is Henoch Shonlein associated with?

Answer 33

IgA nephropathy

Question 34

How does Henoch Schonlein present?

Answer 34

joint pain abdo pain purpuric rash over butocks and lower limb

Question 35

How is Henoch Schonlein managed?

Answer 35

supportive - self limiting | analgesia