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RB Y2 MSK 02 - Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

define vasculitis

A

inflammation of blood vessels

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

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2
Q

why does the clinical presentation vary

A

according to the histological type of inflammation

size of involved blood vessel segment

distribution of involved vessels

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3
Q

how is it classified

A

Chapel Hill Consensus

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4
Q

name the 2 large vessel vaculitis

A

giant cell arthritis

takayasu arthritis

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5
Q

large vessel vasculitis

A

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

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6
Q

what age does large vessel vasculitis occur in

A

GCA - >50 (polymyalgia rheumatica)

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

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7
Q

where does GCA typically affect

A

temporal arteries

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8
Q

who does TA most commonly affect

A

young women in their second and third decades of life

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9
Q

clinical presentation of large vessel vasculitis

A

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

following this claudication symptoms

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10
Q

what can happen if large vessel vasculitis is untreated

A

vascular stenosis and aneurysms

results in reduced pulses and bruits

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11
Q

inflammatory markers for large vessel vasculitis

A

CRP, ESV and PV elevated

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12
Q

what imaging is used in large vessel vasculitis

A

MR angiography can detect thickened walls and stenosis

PE CT shows inc metabolic activity in the larger vessels

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13
Q

treatment of large vessel vasculitis

A

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

steroid sparing agents such as methotrexate and azathioprine may be added

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14
Q

how is small to medium vessel vasculitis divided

A

into ANCA positive and negative

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15
Q

ANCA positive

no granuloma present

A

microscopic polyangiitis

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16
Q

ANCA positive

granuloma present

asthma an eosinophil present

A

churg strauss

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17
Q

ANCA positive

granuloma present

asthma an eosinophil not present

A

wegeners granulomatosis

18
Q

non ANCA

IgA dominant immune deposit

A

henoch-schlnein purpura

19
Q

non ANCA

not IgA dominant immune deposit

serum cryoglobulin

A

cryoglobulinemia

20
Q

non ANCA

not IgA dominant immune deposit

not serum cryoglobulin

A

other

21
Q

what features do many small/medium vessel vasculitis conditions share

A

fever and weight loss

raised non planching purpuric rash

arthraliga/arthritis

mononeuritis multiplex

glomerulonephritis

lung opacities on X ray

22
Q

what is the vasculitis rash like

A

raised non-blanching purpuric rash

23
Q

GPA/Wegeners

A

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

respiratory symptoms - haemoptysis and cavitating lesions on X ray

renal failure

24
Q

what can happen to the nose over time in GPA

A

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

25
Q

what can be seen in the lungs in GPA

A

cavitating opacities

26
Q

what proteins is GPA associated with

A

cANCA and PR3

27
Q

EGPA (Churg Strauss) symptoms

A

late onset asthma, rhinitis and raised peripheral blood eosinophil count

28
Q

what neurological symptoms are seen in EGPA

A

mononeuritis multiplex

29
Q

what type of ANCA for EGPA

A

pANCA (30-70%)

30
Q

what is the most important complication of microscopic polyangiitis

A

(no granulomatous inflammation)

glomerulonephritis - up to 90% patients

31
Q

what antibody is present in MPA

A

pANCA (MPO) in 70-90%

32
Q

which ANCA associated vasculitis is upper airway disease most common in

A

GPA

then EGPA

33
Q

how reliable is ANCA

A

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

34
Q

investigations for small/medium vessel vasculitis

A

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

35
Q

management of ANCA associated vasculitis

A

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

36
Q

Henoch-Schonlein purpura

A

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

37
Q

who does Henoch-Schonlein purpura commonly affect

A

children

38
Q

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

A

history of URT infection

39
Q

common symptoms of Henoch-Schonlein purpura

A

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

40
Q

treatment of Henoch-Schonlein purpura

A

usually self limiting and doesnt require specific treatment

usually settles over the course of weeks to monthhs

41
Q

Cryoglobulinaemia

A

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

RB Y2 MSK 02 - Rheumatology (33 decks)

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