Vasculitis

Question 1

Define vasculitis.

Answer 1

Inflammation of the blood vessels

Question 2

Define primary vasculitis.

Answer 2

Vasculitis that results from an inflammatory response that targets the vessel walls and has no known cause

Question 3

Define secondary vasculitis.

Answer 3

Vasculitis that may be triggered by an infection, drug, toxin or may ocur as a part of another inflammatory disorder or cancer

Question 4

List the 2 main large vessel vasculitis.

Answer 4

Giant cell arteritis
Takayasu arteritis

Question 5

List the 2 main medium vessel vasculitis

Answer 5

Polyarteritis nodosa
Kawasaki disease

Question 6

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

Answer 6

ANCA-associated
Non-ANCA-associated

Question 7

True/False: Takayasu arteritis is more common in males and african populations?

Answer 7

False - More common in females and asian populations

Question 8

What is the typical clinical presentation of Takayasu arteritis?

Answer 8

Early features - fever, malaise, night sweats, weight loss, arthralgia and fatigue
Claudication in upper and lower limbs
Vascular stenosis and aneurysms can occur if untreated

Question 9

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

Answer 9

Polymyalgia rheumatica

Question 10

What is the most common finding in giant cell arteritis?

Answer 10

Carotid bruit

Question 11

What is the mainstay treatment for large vessel vasculitis?

Answer 11

Steroid (40-60mg prednisolone)

Question 12

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

Answer 12

Vessel bifurcations

Question 13

Which group of people is Kawasaki disease seen in?

Answer 13

Children

Question 14

True/False: Hepatitis B is associated with polyarteritis nodosa?

Answer 14

True

Question 15

What are the names for the ANCA +ve small vessel vasculitic disease?

Answer 15

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

Question 16

Which of the ANCA +ve small vessel vasculitic diseases involve granulomatous inflammation?

Answer 16

GPA
EGPA

Question 17

Which of the ANCA +ve small vessel vasculitic diseases are associated with asthma?

Answer 17

EGPA

Question 18

Which of the ANCA +ve small vessel vasculitic diseases are associated with golmerulonephritis?

Answer 18

GPA
MPA

Question 19

True/False: GPA is more common in males?

Answer 19

True

Question 20

When does GPA typically present?

Answer 20

33-35 y,o

Question 21

What affect does GPA have on the nose?

Answer 21

Saddle nose due to cartilage ischaemia

Question 22

What is the typical cutaneous manifestation of GPA?

Answer 22

Palpable purpura

Question 23

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

Answer 23

Impaired blood supply to nerve

Result in wrist/foot drop

Question 24

What is the main difference between GPA and EPGA?

Answer 24

Late onset asthma and high eosinophil count

Question 25

Define ANCA

Answer 25

Anti-neutrophil cytoplasmic antibodies

Question 26

What are the 2 main types of ANCA?

Answer 26

pANCA cANCA

Question 27

Which vasculitis is associated with pANCA?

Answer 27

MPA EGPA

Question 28

Which vasculitis is associated with cANCA?

Answer 28

GPA

Question 29

What antigen does pANCA primarily respond to in MPA?

Answer 29

MPO

Question 30

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

Answer 30

PR3

Question 31

Outline treatment for localised/early systemic ANCA assoc vasculitis

Answer 31

Methotrexate and steroid

Question 32

Outline treatment for generalised/systemic ANCA asso vasculitis

Answer 32

Cyclophosphamide + steroid Ritixumab + steroid Azathioprine

Question 33

Outline treatment for refactory ANCA assoc vasculitis

Answer 33

IV immunoglobulins Rituximab

Question 34

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

Answer 34

Henoch-Schonlein Purpura (HSP)

Question 35

What mediated HSP?

Answer 35

IgA

Question 36

HSP is most common in which age range?

Answer 36

2-11 y o