4: Vasculitis Flashcards

1
Q

What is vasculitis?

What are the consequences of this?

A

Inflammation of blood vessels

Ischaemia and necrosis causing organ dysfunction

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2
Q

Vasculitis can affect ___ size of blood vessel but often presents in distinct ___.

A

any

patterns

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3
Q

Vasculitis tends to be a (single / multi-) system presentation.

A

multi-system

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4
Q

What tends to cause primary vasculitis?

Secondary vasculitis?

A

Primary - autoimmune

Secondary - infection, other stuff

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5
Q

What occurs in blood vessels in vasculitis?

A

Inflammation

Thickening of endothelium

Lumenal narrowing

Ischaemia / claudication…

Necrosis and organ damage

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6
Q

What are the two types of vasculitis you need to know about?

A

Large vessel vasculitis - Takayasu and Giant cell

Small vessel vasculitis - GwP (Wegeners) and eosinophilic (Churg-Strauss)

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7
Q

Which antibody is associated with the small vessel vasculitidies you need to know about?

A

ANCA

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8
Q

What are the constitutional symptoms often seen alongside organ damage in vasculitis?

A

Fever

Malaise

Weight loss

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9
Q

What are the two main types of large vessel vasculitis?

A

Giant cell arteritis

Takayasu arteritis

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10
Q

Takayasu arteritis tends to present in people of which age range?

What about giant cell arteritis?

A

< 40 years old

> 40 years old

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11
Q

large vessel vasculitis - takayasu:

bruit

claud

brachial brachial bp differences

affects aorta, subclav, femoral

A
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12
Q

revise GCA

associated with PMR

end artery to optic nerve

diagnosed by inflam markers and ta biopsy

A
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13
Q

What lights up on a PET CT scan?

A

Metabolically active cells

i.e those involved in inflammation

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14
Q

What sort of scan is useful in large vessel vasculitis?

A

PET CT

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15
Q

PET CT is good for visualising active inflammation - what is used to view structural changes?

A

CT / MRI

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16
Q

How is giant cell arteritis treated?

A

40-60mg prednisolone

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17
Q

How is Takayasu arteritis treated?

A

Steroid

Immunosuppressants

doesn’t burn out as quick as GCA

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18
Q

What percentage of people with GCA also have PMR?

A

40%ish

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19
Q

A higher dose of prednisolone is used in GCA if there is what?

A

Visual loss

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20
Q

What are the ANCA associated small vessel vasculitidies?

A

Wegener’s granulomatosis (now Granulomatosis with polyangiitis)

Churg-Strauss syndrome (now Eosinophilic granulomatosis with polyangiitis)

Microscopic polyangiitis (which affects the kidneys)

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21
Q

GPA is commoner in (men / women) of ___ descent.

A

men

European

22
Q

What are non-specific features of GPA?

A

Constitutional symptoms

23
Q

What are specific features of GWP?

A

ENT SYMPTOMS (nosebleeds, hearing loss)

CHEST SYMPTOMS

RASH

KIDNEY SYMPTOMS (also asymptomatic as in SLE, MUST be screened for, is the most important thing)

24
Q

What test should be carried out immediately in someone with suspected GPA?

A

Urinalysis

25
Q

What happens to the nose in someone with GPA?

A

Saddle nose

cartilage wears away

26
Q

What is seen on CXR in someone with GWP?

A

Cavitating granulomas

27
Q

What does a vasculitic rash look like?

A

Purpuric

Non-blanching

May ulcerate

28
Q

What kind of kidney disease is seen in GPA?

A

Necrotising glomerulonephritis

29
Q

What is a nervous manifestation of GPA?

A

Mononeuritis multiplex

Peripheral nerve stops working - sudden onset foot drop for example

30
Q

What is an ocular manifestation of GWP?

A

Uveitis

Scleritis

31
Q

What should make you suspect EGWP as opposed to GWP?

A

Late onset asthma

High eosinophil count

32
Q

What are the key features which would make you suspect EGWP?

A

Asthma

Eosinophilia

Proof of vasculitis w/ eosinophils on biopsy

33
Q

Are ENT symptoms more a feature of GWP or EGWP?

A

GWP

34
Q

What does ANCA stand for?

A

Anti-neutrophil cytoplasmic antibody

35
Q

What are the two types of ANCA?

A

cANCA

pANCA

36
Q

What is cANCA associated with?

A

GWP

37
Q

What is pANCA associated with?

A

EGWP

Microscopic polyangiitis

38
Q

What is the antibody raised in active GWP?

A

PR3

39
Q

What is the antibody raised in active EGPA?

A

MPO

40
Q

so to recap

GPA - cANCA and PR3

EGPA - pANCA and MPO

A
41
Q

When vasculitis is active, complement levels (increase / decrease).

A

decrease

as they are consumed

42
Q

How is small vessel vasculitis treated?

A

IV steroid

IV cyclophosphamide

43
Q

Vasculitis patients must be treated ___ and ___.

A

early

aggressively

44
Q

Even if urinanalysis normal, what must be done in a new patient presenting with vasculitis?

Why?

A

Renal biopsy

Kidney involvement gives overall disease severity

45
Q

Which disease is usually provoked by infection, self-limiting, and mediated by IgA?

A

Henoch-Schonlein purpura

46
Q

What is the presentation of HSP?

A

Rash

+/- GI symptoms

+/- Cough

47
Q

The majority of HSP cases are seen in ___.

A

children

48
Q

What tends to cause HSP?

A

Group A Strep**. infection

49
Q

Where is the rash of HSP seen?

A

Feet, legs, buttocks

50
Q

What test should still be carried out in those with HSP?

A

Urinalysis

51
Q

How long does HSP typically last?

A

2 months

52
Q

Can HSP relapse?

A

Yes