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Flashcards in Vasculitis Deck (42):
1

What is vasculitis?

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

2

True or False
Vasculitis only affects arteries and veins

False.
Venules, arterioles and capillaries can all be affected too

3

What are the two types of vasculitis?

Primary and secondary

4

What can trigger secondary vasculitis?

Infection, drugs or a toxin

5

What is the pathogenesis of vasculitis?

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

6

How is vasculitis classified?

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

7

What is the classical presentation of vasculitis?

Depends very much on where it affects.
Fever, malaise, fatigue and weight loss are all common,

8

What are the common causes of large vessel vasculitis?

Giant cell arteritis
Takaysu arteritis

9

What condition has a link with giant cell arteritis?

Polymyalgia rheumatica

10

Who is more likely to develop Takaysu arteritis?

Females under 40.
More common in Asians

11

Where does giant cell arteritis affect?

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

12

What clinical findings may be present in large vessel vasculitis?

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

13

What are the classical symptoms of temporal arteritis?

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

14

What is the major concern with temporal arteritis?

risk of blindess due to ischaemia of optic nerve

15

What investigations would you do if you suspected TA?

Inflammatory markers -ESR, CRP, PV
MR angiogram

16

True or False
Temporal artery biopsy is the definitive investigation

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

17

What is the management of TA?

40mg prednisolone
DMARDs if necessary

18

What is Kawasaki disease?

Medium vessel vasculitis
Kids

19

What is polyarteritis nodosa?

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

20

What is granulomatois with polyangitis (GPA)?

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

21

What did GPA used to be called?

Wegner's syndrome

22

Who is GPA most common in?

Slightly more common in males. 35-55

23

What are the features of GPA?

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

24

What is the medical term for nosebleeds?

Epistaxis

25

What respiratory features are present in GPA?

Cough, haemoptysis, pulmonary infiltrates

26

What is the difference in GPA and EGPA?

EGPA is eosinophilic and presents with asthma

27

What did EGPA used to be called?

Churg-Strauss

28

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

Inflammatory markers
CXR
ANCA
Urinalysis

29

What is the treatment cascade for (E)GPA?

Methotrexate &; steroids if localised

Cyclophosphamide &; steroids if systemic

IV immunoglobulin if progressive and unresponsive

30

What is Henoch-Schonlein purpura?

Acute IgA mediated disorder

31

Who normally gets Henoch- Schonlein purpura?

Majority in children 2-11

32

Where does Henoch- Schonlein purpura normally affect?

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

33

What normally comes before HSP?

URTI, GI infection.
Normally group A strep.

34

What does HSP present as?

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

35

What is the management for HSP?

resolves in 8 weeks
urinalysis essential to screen for renal involvement

36

How can skin present in GPA?

Palpable, non-blanching purpuras

37

What renal involvement can develop in GPA?

Necrotising glomerulonephritis so do urinalysis

38

What is ANCA?

Ab against proteins in the cytoplasmic against neutrophils

39

What are the two types of ANCA?

cANCA
pANCA

40

Which condition is associated with cANCA?

GPA

41

Which ANCA is PR3 associated with?

cANCA

42

Which ANCA is MPO associated with?

pANCA