4: Vasculitis

Question 1

What is vasculitis?

What are the consequences of this?

Answer 1

Inflammation of blood vessels

Ischaemia and necrosis causing organ dysfunction

Question 2

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

Answer 2

any

patterns

Question 3

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

Answer 3

multi-system

Question 4

What tends to cause primary vasculitis?

Secondary vasculitis?

Answer 4

Primary - autoimmune

Secondary - infection, other stuff

Question 5

What occurs in blood vessels in vasculitis?

Answer 5

Inflammation

Thickening of endothelium

Lumenal narrowing

Ischaemia / claudication…

Necrosis and organ damage

Question 6

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

Answer 6

Large vessel vasculitis - Takayasu and Giant cell

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

Question 7

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

Answer 7

ANCA

Question 8

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

Answer 8

Fever

Malaise

Weight loss

Question 9

What are the two main types of large vessel vasculitis?

Answer 9

Giant cell arteritis

Takayasu arteritis

Question 10

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

What about giant cell arteritis?

Answer 10

< 40 years old

> 40 years old

Question 11

large vessel vasculitis - takayasu:

bruit

claud

brachial brachial bp differences

affects aorta, subclav, femoral

Question 12

revise GCA

associated with PMR

end artery to optic nerve

diagnosed by inflam markers and ta biopsy

Question 13

What lights up on a PET CT scan?

Answer 13

Metabolically active cells

i.e those involved in inflammation

Question 14

What sort of scan is useful in large vessel vasculitis?

Answer 14

PET CT

Question 15

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

Answer 15

CT / MRI

Question 16

How is giant cell arteritis treated?

Answer 16

40-60mg prednisolone

Question 17

How is Takayasu arteritis treated?

Answer 17

Steroid

Immunosuppressants

doesn’t burn out as quick as GCA

Question 18

What percentage of people with GCA also have PMR?

Answer 18

40%ish

Question 19

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

Answer 19

Visual loss

Question 20

What are the ANCA associated small vessel vasculitidies?

Answer 20

Wegener’s granulomatosis (now Granulomatosis with polyangiitis)

Churg-Strauss syndrome (now Eosinophilic granulomatosis with polyangiitis)

Microscopic polyangiitis (which affects the kidneys)

Question 21

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

Answer 21

men

European

Question 22

What are non-specific features of GPA?

Answer 22

Constitutional symptoms

Question 23

What are specific features of GWP?

Answer 23

ENT SYMPTOMS (nosebleeds, hearing loss)

CHEST SYMPTOMS

RASH

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

Question 24

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

Answer 24

Urinalysis

Question 25

What happens to the nose in someone with GPA?

Answer 25

**Saddle nose** cartilage wears away

Question 26

What is seen on CXR in someone with GWP?

Answer 26

**Cavitating granulomas**

Question 27

What does a vasculitic rash look like?

Answer 27

**Purpuric** **Non-blanching** May ulcerate

Question 28

What kind of kidney disease is seen in GPA?

Answer 28

**Necrotising glomerulonephritis**

Question 29

What is a nervous manifestation of GPA?

Answer 29

**Mononeuritis multiplex** Peripheral nerve stops working - sudden onset foot drop for example

Question 30

What is an ocular manifestation of GWP?

Answer 30

**Uveitis** **Scleritis**

Question 31

What should make you suspect **EGWP** as opposed to **GWP****?**

Answer 31

**Late onset asthma** **High eosinophil count**

Question 32

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

Answer 32

**Asthma** **Eosinophilia** **Proof of vasculitis w/ eosinophils on biopsy**

Question 33

Are ENT symptoms more a feature of **GWP** or **EGWP?**

Answer 33

**GWP**

Question 34

What does ANCA stand for?

Answer 34

**Anti-neutrophil cytoplasmic antibody**

Question 35

What are the two types of ANCA?

Answer 35

**cANCA** **pANCA**

Question 36

What is cANCA associated with?

Answer 36

**GWP**

Question 37

What is pANCA associated with?

Answer 37

**EGWP** **Microscopic polyangiitis**

Question 38

What is the antibody raised in active GWP?

Answer 38

**PR3**

Question 39

What is the antibody raised in active EGPA?

Answer 39

**MPO**

Question 40

so to recap GPA - cANCA and PR3 EGPA - pANCA and MPO

Question 41

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

Answer 41

**decrease** as they are consumed

Question 42

How is small vessel vasculitis treated?

Answer 42

**IV steroid** **IV cyclophosphamide**

Question 43

Vasculitis patients must be treated ___ and \_\_\_.

Answer 43

**early** **aggressively**

Question 44

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

Answer 44

Renal biopsy Kidney involvement gives overall disease severity

Question 45

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

Answer 45

**Henoch-Schonlein purpura**

Question 46

What is the presentation of HSP?

Answer 46

**Rash** +/- GI symptoms +/- Cough

Question 47

The majority of HSP cases are seen in \_\_\_.

Answer 47

**children**

Question 48

What tends to cause HSP?

Answer 48

**Group A *Strep**.* infection**

Question 49

Where is the rash of HSP seen?

Answer 49

**Feet, legs, buttocks**

Question 50

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

Answer 50

**Urinalysis**

Question 51

How long does HSP typically last?

Answer 51

**2 months**

Question 52

Can HSP relapse?

Answer 52

**Yes**