Vasculitis

Question 1

How does primary vasculitis arise?

Answer 1

Inflammatory response (can be autoimmune) that occurs in the walls of blood vessels, causing damage and reduced blood flow

Question 2

List the 2 main large vessel vasculitis

Answer 2

Giant cell arteritis

Takayasu arteritis

Question 3

List the 2 main medium vessel vasculitis

Answer 3

Polyarteritis nodosa

Kawasaki disease

Question 4

Small vessel vasculitis can be generally divided into which 2 groups?

Answer 4

ANCA-associated

Non-ANCA-associated

Question 5

Takayasu arteritis is commoner in males and in Asian populations. True/False?

Answer 5

False

Commoner in females and Asian populations

Question 6

Giant cell arteritis affects those over 50 and is associated with which other disease?

Answer 6

Polymyalgia rheumatica

Question 7

What is the most common finding in giant cell arteritis?

Answer 7

Carotid bruit

Question 8

What is the mainstay of treatment for large vessel vasculitis?

Answer 8

Steroid (40-60mg prednisolone)

Question 9

Which group of people is Kawasaki disease seen in?

Answer 9

Children

Question 10

Which part of the artery is usually affected in polyarteritis nodosa?

Answer 10

Vessel bifurcations

Question 11

Which hepatitis is associated with polyarteritis nodosa?

Answer 11

Hepatitis B

Question 12

What are the old names for the ANCA-positive small vessel vasculitic diseases?

Answer 12

Wegener’s granulomatosis
Churg-Strauss syndrome
Microscopic polyangitis

Question 13

What are the new names for the ANCA-positive small vessel vasculitic diseases?

Answer 13

Granulomatosis with polyangitis (GPA)
Eosinophilic granulomatosis with polyangitis (EGPA)
Microscopic polyangitis (MPA)

Question 14

Which of the 3 main ANCA-positive small vessel vasculitic diseases involve granulomatous inflammation?

Answer 14

GPA

EGPA

Question 15

Which ANCA-positive small vessel vasculitic disease is associated with asthma?

Answer 15

EGPA

Question 16

Which ANCA-positive small vessel vasculitic diseases are associated with glomerulonephritis?

Answer 16

GPA

MPA

Question 17

GPA is more common in males. True/False?

Answer 17

True

Question 18

When does GPA typically present?

Answer 18

35-55yrs old

Question 19

What effect does GPA have on the nose?

Answer 19

Saddle nose due to cartilage ischaemia

Question 20

What is the usual cutaneous manifestation of GPA?

Answer 20

Palpable purpura

Question 21

What is mononeuritis multiplex and what effect can it have in GPA?

Answer 21

Impaired blood supply to nerve

Wrist/foot drop

Question 22

What is the main difference between GPA and EGPA?

Answer 22

Late onset asthma

High eosinophil count

Question 23

What are the 2 main types of ANCA?

Answer 23

pANCA

cANCA

Question 24

Which vasculitis is associated with pANCA?

Answer 24

MPA

EGPA

Question 25

Which vasculitis is associated with cANCA?

Answer 25

GPA

Question 26

What is the antigen that pANCA primarily responds to in MPA?

Answer 26

MPO

Question 27

What is the antigen that cANCA primarily responds to in GPA?

Answer 27

PR3

Question 28

Outline treatment for localised/early systemic AAV

Answer 28

Methotrexate + steroid

Question 29

Outline treatment for generalised/systemic AAV

Answer 29

Cyclophosphamide + steroid Ritixumab + steroid Azathioprine

Question 30

Outline treatment for refractory AAV

Answer 30

IV immunoglobulins | Rituximab

Question 31

What is the main non-ANCA-associated small vessel vasculitic disease?

Answer 31

Henoch-Schonlein Purpura (HSP)

Question 32

Which Ig mediates HSP?

Answer 32

IgA

Question 33

HSP is most common in which age range?

Answer 33

2-11 yrs old