Vasculitis

Question 1

What is vasculitis?

Answer 1

Inflammation of blood vessels, with ischaemia, necrosis and organ inflammation

Question 2

True or False

Vasculitis only affects arteries and veins

Answer 2

False.

Venules, arterioles and capillaries can all be affected too

Question 3

What are the two types of vasculitis?

Answer 3

Primary and secondary

Question 4

What can trigger secondary vasculitis?

Answer 4

Infection, drugs or a toxin

Question 5

What is the pathogenesis of vasculitis?

Answer 5

Something causes dendritic cells to release cytokines which causes a cascade of WBC infiltration which leads to endothelial damage

Question 6

How is vasculitis classified?

Answer 6

Large vessel,
medium vessel
ANCA-associated small vessel
immune complex small vessel

Question 7

What is the classical presentation of vasculitis?

Answer 7

Depends very much on where it affects.

Fever, malaise, fatigue and weight loss are all common,

Question 8

What are the common causes of large vessel vasculitis?

Answer 8

Giant cell arteritis

Takaysu arteritis

Question 9

What condition has a link with giant cell arteritis?

Answer 9

Polymyalgia rheumatica

Question 10

Who is more likely to develop Takaysu arteritis?

Answer 10

Females under 40.

More common in Asians

Question 11

Where does giant cell arteritis affect?

Answer 11

Typically temporal arteries but can also be in aorta and other great vessels

Question 12

What clinical findings may be present in large vessel vasculitis?

Answer 12

Bruit, especially at carotids
Blood pressure different at extremities
Claudication
Hypertension

Question 13

What are the classical symptoms of temporal arteritis?

Answer 13

Jaw claudication
Unilateral, temporal headache
Prominent, non-pulsating temporal arteries.

Question 14

What is the major concern with temporal arteritis?

Answer 14

risk of blindess due to ischaemia of optic nerve

Question 15

What investigations would you do if you suspected TA?

Answer 15

Inflammatory markers -ESR, CRP, PV

MR angiogram

Question 16

True or False

Temporal artery biopsy is the definitive investigation

Answer 16

False.

Skip lesions occur so a negative biopsy does not rule out TA

Question 17

What is the management of TA?

Answer 17

40mg prednisolone

DMARDs if necessary

Question 18

What is Kawasaki disease?

Answer 18

Medium vessel vasculitis

Kids

Question 19

What is polyarteritis nodosa?

Answer 19

Medium vessel vasculitis
necrotising inflammatory lesion at bifurcations
Affects skin, gut and kidneys

Question 20

What is granulomatois with polyangitis (GPA)?

Answer 20

Granulomatous inflammation of respiratory tract and sm. & med. vessels.
Glomerulonephritis common.

Question 21

What did GPA used to be called?

Answer 21

Wegner’s syndrome

Question 22

Who is GPA most common in?

Answer 22

Slightly more common in males. 35-55

Question 23

What are the features of GPA?

Answer 23

Constitutional symptoms and arthralgia
Lots of ENT features- sinusitis, ulcers, saddle nose, nosebleeds
Also conjunctivitis, uveitis and deafness

Question 24

What is the medical term for nosebleeds?

Answer 24

Epistaxis

Question 25

What respiratory features are present in GPA?

Answer 25

Cough, haemoptysis, pulmonary infiltrates

Question 26

What is the difference in GPA and EGPA?

Answer 26

EGPA is eosinophilic and presents with asthma

Question 27

What did EGPA used to be called?

Answer 27

Churg-Strauss

Question 28

If you suspected a (E)GPA what investigations would you do?

Answer 28

Inflammatory markers CXR ANCA Urinalysis

Question 29

What is the treatment cascade for (E)GPA?

Answer 29

Methotrexate &; steroids if localised Cyclophosphamide &; steroids if systemic IV immunoglobulin if progressive and unresponsive

Question 30

What is Henoch-Schonlein purpura?

Answer 30

Acute IgA mediated disorder

Question 31

Who normally gets Henoch- Schonlein purpura?

Answer 31

Majority in children 2-11

Question 32

Where does Henoch- Schonlein purpura normally affect?

Answer 32

Small vessels of skin, GI tract, kidneys and joints.

Question 33

What normally comes before HSP?

Answer 33

URTI, GI infection. | Normally group A strep.

Question 34

What does HSP present as?

Answer 34

Purpuric rash over buttocks and lower limbs Colicky abdo pain Bloody diarrhoea Joint pain

Question 35

What is the management for HSP?

Answer 35

resolves in 8 weeks | urinalysis essential to screen for renal involvement

Question 36

How can skin present in GPA?

Answer 36

Palpable, non-blanching purpuras

Question 37

What renal involvement can develop in GPA?

Answer 37

Necrotising glomerulonephritis so do urinalysis

Question 38

What is ANCA?

Answer 38

Ab against proteins in the cytoplasmic against neutrophils

Question 39

What are the two types of ANCA?

Answer 39

cANCA | pANCA

Question 40

Which condition is associated with cANCA?

Answer 40

GPA

Question 41

Which ANCA is PR3 associated with?

Answer 41

cANCA

Question 42

Which ANCA is MPO associated with?

Answer 42

pANCA