Vasculitis Flashcards

1
Q

What is vasculitis?

A

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

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2
Q

True or False

Vasculitis only affects arteries and veins

A

False.

Venules, arterioles and capillaries can all be affected too

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3
Q

What are the two types of vasculitis?

A

Primary and secondary

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4
Q

What can trigger secondary vasculitis?

A

Infection, drugs or a toxin

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5
Q

What is the pathogenesis of vasculitis?

A

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

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6
Q

How is vasculitis classified?

A

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

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7
Q

What is the classical presentation of vasculitis?

A

Depends very much on where it affects.

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

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8
Q

What are the common causes of large vessel vasculitis?

A

Giant cell arteritis

Takaysu arteritis

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9
Q

What condition has a link with giant cell arteritis?

A

Polymyalgia rheumatica

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10
Q

Who is more likely to develop Takaysu arteritis?

A

Females under 40.

More common in Asians

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11
Q

Where does giant cell arteritis affect?

A

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

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12
Q

What clinical findings may be present in large vessel vasculitis?

A

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

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13
Q

What are the classical symptoms of temporal arteritis?

A

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

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14
Q

What is the major concern with temporal arteritis?

A

risk of blindess due to ischaemia of optic nerve

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15
Q

What investigations would you do if you suspected TA?

A

Inflammatory markers -ESR, CRP, PV

MR angiogram

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16
Q

True or False

Temporal artery biopsy is the definitive investigation

A

False.

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

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17
Q

What is the management of TA?

A

40mg prednisolone

DMARDs if necessary

18
Q

What is Kawasaki disease?

A

Medium vessel vasculitis

Kids

19
Q

What is polyarteritis nodosa?

A

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

20
Q

What is granulomatois with polyangitis (GPA)?

A

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

21
Q

What did GPA used to be called?

A

Wegner’s syndrome

22
Q

Who is GPA most common in?

A

Slightly more common in males. 35-55

23
Q

What are the features of GPA?

A

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

24
Q

What is the medical term for nosebleeds?

A

Epistaxis

25
Q

What respiratory features are present in GPA?

A

Cough, haemoptysis, pulmonary infiltrates

26
Q

What is the difference in GPA and EGPA?

A

EGPA is eosinophilic and presents with asthma

27
Q

What did EGPA used to be called?

A

Churg-Strauss

28
Q

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

A

Inflammatory markers
CXR
ANCA
Urinalysis

29
Q

What is the treatment cascade for (E)GPA?

A

Methotrexate &; steroids if localised

Cyclophosphamide &; steroids if systemic

IV immunoglobulin if progressive and unresponsive

30
Q

What is Henoch-Schonlein purpura?

A

Acute IgA mediated disorder

31
Q

Who normally gets Henoch- Schonlein purpura?

A

Majority in children 2-11

32
Q

Where does Henoch- Schonlein purpura normally affect?

A

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

33
Q

What normally comes before HSP?

A

URTI, GI infection.

Normally group A strep.

34
Q

What does HSP present as?

A

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

35
Q

What is the management for HSP?

A

resolves in 8 weeks

urinalysis essential to screen for renal involvement

36
Q

How can skin present in GPA?

A

Palpable, non-blanching purpuras

37
Q

What renal involvement can develop in GPA?

A

Necrotising glomerulonephritis so do urinalysis

38
Q

What is ANCA?

A

Ab against proteins in the cytoplasmic against neutrophils

39
Q

What are the two types of ANCA?

A

cANCA

pANCA

40
Q

Which condition is associated with cANCA?

A

GPA

41
Q

Which ANCA is PR3 associated with?

A

cANCA

42
Q

Which ANCA is MPO associated with?

A

pANCA