Vasculitis

Question 1

define vasculitis

Answer 1

inflammation of blood vessels

may result in vessel wall thickening, stenosis and occlusion with subsequent ischaemia

Question 2

why does the clinical presentation vary

Answer 2

according to the histological type of inflammation

size of involved blood vessel segment

distribution of involved vessels

Question 3

how is it classified

Answer 3

Chapel Hill Consensus

Question 4

name the 2 large vessel vaculitis

Answer 4

giant cell arthritis

takayasu arthritis

Question 5

large vessel vasculitis

Answer 5

applies to primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches

Question 6

what age does large vessel vasculitis occur in

Answer 6

GCA - >50 (polymyalgia rheumatica)

Takayasu <50 (women in 20s and 30s)

Question 7

where does GCA typically affect

Answer 7

temporal arteries

Question 8

who does TA most commonly affect

Answer 8

young women in their second and third decades of life

Question 9

clinical presentation of large vessel vasculitis

Answer 9

early non specific features eg fever, weight loss, night sweats, malaise, arthralgia and fatigue

following this claudication symptoms

Question 10

what can happen if large vessel vasculitis is untreated

Answer 10

vascular stenosis and aneurysms

results in reduced pulses and bruits

Question 11

inflammatory markers for large vessel vasculitis

Answer 11

CRP, ESV and PV elevated

Question 12

what imaging is used in large vessel vasculitis

Answer 12

MR angiography can detect thickened walls and stenosis

PE CT shows inc metabolic activity in the larger vessels

Question 13

treatment of large vessel vasculitis

Answer 13

corticosteroids - starting a 40/60mg prednisolone and gradually reducing

steroid sparing agents such as methotrexate and azathioprine may be added

Question 14

how is small to medium vessel vasculitis divided

Answer 14

into ANCA positive and negative

Question 15

ANCA positive

no granuloma present

Answer 15

microscopic polyangiitis

Question 16

ANCA positive

granuloma present

asthma an eosinophil present

Answer 16

churg strauss

Question 17

ANCA positive

granuloma present

asthma an eosinophil not present

Answer 17

wegeners granulomatosis

Question 18

non ANCA

IgA dominant immune deposit

Answer 18

henoch-schlnein purpura

Question 19

non ANCA

not IgA dominant immune deposit

serum cryoglobulin

Answer 19

cryoglobulinemia

Question 20

non ANCA

not IgA dominant immune deposit

not serum cryoglobulin

Answer 20

other

Question 21

what features do many small/medium vessel vasculitis conditions share

Answer 21

fever and weight loss

raised non planching purpuric rash

arthraliga/arthritis

mononeuritis multiplex

glomerulonephritis

lung opacities on X ray

Question 22

what is the vasculitis rash like

Answer 22

raised non-blanching purpuric rash

Question 23

GPA/Wegeners

Answer 23

ENT symptoms common - nose bleed, deafness, recurrent sinusitis and nasal crusting

respiratory symptoms - haemoptysis and cavitating lesions on X ray

renal failure

Question 24

what can happen to the nose over time in GPA

Answer 24

recurrent sinusitis and nasal crusting can lead to collapse of the nose

Question 25

what can be seen in the lungs in GPA

Answer 25

cavitating opacities

Question 26

what proteins is GPA associated with

Answer 26

cANCA and PR3

Question 27

EGPA (Churg Strauss) symptoms

Answer 27

late onset asthma, rhinitis and raised peripheral blood eosinophil count

Question 28

what neurological symptoms are seen in EGPA

Answer 28

mononeuritis multiplex

Question 29

what type of ANCA for EGPA

Answer 29

pANCA (30-70%)

Question 30

what is the most important complication of microscopic polyangiitis

Answer 30

(no granulomatous inflammation) glomerulonephritis - up to 90% patients

Question 31

what antibody is present in MPA

Answer 31

pANCA (MPO) in 70-90%

Question 32

which ANCA associated vasculitis is upper airway disease most common in

Answer 32

GPA then EGPA

Question 33

how reliable is ANCA

Answer 33

is negative in a proportion of all these conditions so cannot be relied upon for a diagnosis

Question 34

investigations for small/medium vessel vasculitis

Answer 34

ESR, PV, CRP raised anaemic of chronic disease is common U and E for renal involvement ANCA urinalysis (looking for renal vasculitis) CXR biopsy of affected area

Question 35

management of ANCA associated vasculitis

Answer 35

most cases of ANCA associated vasculitis require treatment with **IV steroids** and **cyclophosphamide** due to their agressive disease course there are some situations eg GPA localised in the nose where other options would be considered

Question 36

Henoch-Schonlein purpura

Answer 36

acute IgA mediated disorder characterised by generalised vasculitis involving the small vessels of the skin, the GI tract, the kidneys, joints, and the lungs and CNS system rarely

Question 37

who does Henoch-Schonlein purpura commonly affect

Answer 37

children

Question 38

what commonly predates Henoch-Schonlein purpura symptoms by a few weeks

Answer 38

history of URT infection

Question 39

common symptoms of Henoch-Schonlein purpura

Answer 39

purpuric rash over the buttocks and lower limbs, abdominal pain and vomiting and joint pain

Question 40

treatment of Henoch-Schonlein purpura

Answer 40

usually self limiting and doesnt require specific treatment usually settles over the course of weeks to monthhs

Question 41

**Cryoglobulinaemia**

Answer 41

symptoms of peripheral rash, ulceration and Raynaud's are typical