vasculitis Flashcards

1
Q

what is vasculitis

A

autoimmune inflam of blood vessels –> thickness and stenosis

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2
Q

what are serious side effects of vasculitis

A

ischaemia, necrosis and organ inflam

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3
Q

what is the pathogenesis of primary vasculitis

A

inflam response in vessel walls become leaky and narrow, thrombus and fibrin deposits –> reduces flow

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4
Q

what is secondary vasculitis

A

triggered by infection, drugs, toxins or another inflam disorder eg cancer

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5
Q

what are the 2 main types of large vessel vasculitis

A

Takayasu arteritis (TA), giant cell arteritis (GCA)

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6
Q

who commonly gets TA

A

asian women under 40

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7
Q

what vessels does TA commonly affect

A

arteries of the aorta

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8
Q

what causes large vessel vasculitis

A

granulomatous infiltration of large vessel walls

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9
Q

what are common symptoms associated with large vessel vasculitis

A

bruits (carotid), reduced pulses, low grade fever, malaise, night sweats, BP difference in extremities

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10
Q

what is the most common form of systemic vasculitis

A

giant cell arteritis

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11
Q

who commonly gets giant cell arteritis

A

older patients with polymyalgia rheumatica

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12
Q

what is the pathogenesis of giant cell arteritis

A

inflamm of tunica intima, media and adventitia by lymphocytes + macrophages causing granulomas

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13
Q

what are the symptoms of giant cell arteritis

A

headache (occipital and temporal), scalp tenderness, jaw claudication, visual loss, tender, non-palpable arteries, fever, malaise, fatigue

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14
Q

what investigations are done for giant cell arteritis and TA

A

temporal artery biopsy (gold), bloods; PV, CRP. MR angiogram, CT

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15
Q

what is the treatment of giant cell arteritis

A

40-60mg pred - 60mg if visual disturbance

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16
Q

what subgroups is small vessel vasculitis divided into

A

ANCA +ive and ANCA -ive

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17
Q

what are the ANCA +ive small vessel vasculitis

A

granulomatosis polyangiitis (GPA), Eosinophillic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA)

18
Q

what are the ANCA -ive small vessel vasculitis

A

Henoch-Schnlein purpura

19
Q

who commonly gets GPA

A

white men 30-50s

20
Q

what body systems are commonly affected in GPA

A

nasopharynx, lungs’ kidneys

21
Q

what symptoms are present in GPA

A

sinusitis, ulcers, SADDLE NOSE, nose bleeds, otitis media, cough/ haemoptysis, palpable purpura, eye problems

22
Q

what autoantibodies are present in GPA

A

cANCA and anti-PR3

23
Q

what is the GPA diagnostic criteria (5)

A

nasal/ oral inflamm (ulcers/ discharge), chest imaging with nodules, urinary sediment, granulomatous inflamm on biopsy, cANCA + anti-PR3

24
Q

what is the main difference between EGPA and GPA

A

EGPA is symptoms of late onset asthma and high eosinophil count

25
what is the diagnostic criteria for EGPA (6)
(4+) asthma, eosinophilia, histological proof of eosinophilia, paranasal sinusitis, puml infiltrates, polyneuropathy
26
what antibodies are present in EGPA
pANCA and anti-MPO
27
how is MPA different to GPA and EGPA
doesn't affect nasopharynx, no granulomas present
28
what does MPA usually cause
glomerulonephritis
29
what antibodies are present in MPA
pANCA and anti-MPO
30
what are the investigations of ANCA +ive small vessel vasculitis
bloods: PV, CRP, FBC/ U+E's, ANCA, CXR
31
what is the management + criteria of localised/ early ANCA +ive small vessel vasculitis
U/LRTI with non life threatening organ involvement: methotrexate + steroids
32
what is the management + criteria of generalised/ systemic ANCA +ive small vessel vasculitis
renal/ organ threatening: cyclophosphamide + steroids OR rituximab + steroids
33
what is the management + criteria of refractory ANCA +ive small vessel vasculitis
progressive/ unresponsive to steroids: IV immunoglobins + rituximab
34
what is HSP
ANCA -ive, IgA mediated vasculitis
35
who commonly gets HSP
children
36
what normally preceeds HSP
URTI, pharyngeal infection or GI infections (GAS organisms)
37
what are the main symptoms of HSP (4)
purpura, abdo pain, arthritis, renal involvement (50%), lungs
38
describe the purpura of HSP
purpuric (purple) rash over buttocks and lower limb (punch out legions)
39
what abdo pain can come with HSP
colicky, bloody diarrhoea, vomiting
40
what management is there for HSP
normally self-limiting <8 weeks, urinalysis for renal damage