Vasculitis

Question 1

Which condition is cANCA positive and associated with anti-PR3?

Answer 1

GPA

Question 2

How is Henoch Schonlein purpura treated?

Answer 2

This is self limiting, and will settle over weeks-months

Question 3

GPA can be diagnosed if at least 2 of what 4 features are present?

Answer 3

Nasal/oral inflammation, abnormal chest radiograph, urinary sediment, granulomatous inflammation on biopsy

Question 4

What are some common symptoms of GCA?

Answer 4

Headache, visual changes, jaw claudication, scalp tenderness

Question 5

If someone has features of temporal arteritis, what investigation is indicated?

Answer 5

Temporal artery biopsy

Question 6

What types of imaging may be useful for large vessel vasculitis?

Answer 6

MR angiogram or PET CT

Question 7

What are some features which vary with disease activity in small vessel vasculitis?

Answer 7

ANCA, anti-PR3, anti-MPO and C3/4

Question 8

MPA is positive for which type of ANCA?

Answer 8

pANCA

Question 9

How is large vessel vasculitis treated?

Answer 9

Initially with 40-60mg prednisolone, then DMARDs e.g. methotrexate/azathioprine may be considered

Question 10

Apart from the rash, what are some other potential features of Henoch Schonlein purpura?

Answer 10

Colicky abdominal pain, vomiting and joint pain/swelling

Question 11

Who is Takayasu arteritis most commonly seen in?

Answer 11

Usually females aged < 50, often Asian origin

Question 12

Which vessels are most commonly involved in Takayasu arteritis?

Answer 12

Aorta, femoral and subclavian arteries

Question 13

What investigations should be used for diagnosis of ANCA vasculitis?

Answer 13

Routine bloods, antibody screen, urinalysis, CXR and biopsy of an affected area

Question 14

If large vessel vasculitis is left untreated- what can this lead to?

Answer 14

Claudicant symptoms in the limbs, reduced pulses and bruits

Question 15

ENT symptoms are common in GPA. What are some examples?

Answer 15

Nasal crusting, epistaxis, deafness, recurrent sinusitis

Question 16

What are some neurological features of small vessel vasculitis?

Answer 16

Mononeuritis multiplex (causing symptoms e.g. foot/wrist drop) and a glove and stocking distribution peripheral neuropathy

Question 17

What are some typical features of all kinds of small vessel vasculitis?

Answer 17

Purpuric rash, mononeuritis multiplex, glomerulonephritis, lung opacities

Question 18

Who does Henoch Schonlein purpura usually occur in? What typically precedes its occurrance?

Answer 18

Usually occurs in children, with a history of a recent URTI (usually GAS)

Question 19

EGPA presents similarly to GPA- what are some features that characterise EGPA?

Answer 19

Late onset asthma, rhinitis and a peripheral blood eosinophil count of > 10%

Question 20

Kawasaki disease can cause vasculitis of various vessels, but which do we worry about most?

Answer 20

The coronary arteries

Question 21

What is an important feature of an FBC seen in ANCA vasculitis?

Answer 21

Anaemia of chronic disease

Question 22

What investigation should be done in everyone presenting with Henoch Schonlein purpura and why?

Answer 22

Urinalysis, as renal involvement occurs in 50% of cases

Question 23

‘Saddle nose’ is a sign associated with which type of vasculitis?

Answer 23

GPA

Question 24

Who is Kawasaki disease usually seen in?

Answer 24

Children aged < 5

Question 25

Why does a negative temporal artery biopsy not mean an individual does not have GCA?

Answer 25

There are skip lesions, so a negative biopsy does not rule out the condition

Question 26

What are the two main types of large vessel vasculitis?

Answer 26

Giant cell arteritis and Takayasu arteritis

Question 27

Henoch Schonlein purpura is a type of small vessel vasculitis mediated by what?

Answer 27

IgA

Question 28

Cases of ANCA vasculitis with some degree of organ involvement should be managed how?

Answer 28

IV steroids and cyclophosphamide (stepped down to another DMAD after 3-6 months)

Question 29

Who does giant cell arteritis typically affect? Which vessel is most commonly involved?

Answer 29

Usually involves in the temporal artery in adults aged > 50

Question 30

What is the most common feature of Henoch Schonlein purpura?

Answer 30

Purpuric, non-blanching rash over the buttocks and lower limbs

Question 31

Describe what is meant by large vessel vasculitis?

Answer 31

Chronic granulomatous inflammation of the aorta and its major branches

Question 32

If ANCA vasculitis has no systemic involvement or constitutional symptoms, how should it be treated?

Answer 32

Oral steroids and methotrexate

Question 33

When should treatment for GCA be started?

Answer 33

As soon as the condition is suspected

Question 34

Which type of vasculitis is associated with hepatitis B?

Answer 34

Polyarteritis nodosa

Question 35

Which condition is pANCA positive and associated with anti-MPO?

Answer 35

EGPA

Question 36

What are the two main types of medium vessel vasculitis?

Answer 36

Kawasaki disease and polyarteritis nodosa