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
Q

what is the diagnostic criteria for EGPA (6)

A

(4+) asthma, eosinophilia, histological proof of eosinophilia, paranasal sinusitis, puml infiltrates, polyneuropathy

26
Q

what antibodies are present in EGPA

A

pANCA and anti-MPO

27
Q

how is MPA different to GPA and EGPA

A

doesn’t affect nasopharynx, no granulomas present

28
Q

what does MPA usually cause

A

glomerulonephritis

29
Q

what antibodies are present in MPA

A

pANCA and anti-MPO

30
Q

what are the investigations of ANCA +ive small vessel vasculitis

A

bloods: PV, CRP, FBC/ U+E’s, ANCA, CXR

31
Q

what is the management + criteria of localised/ early ANCA +ive small vessel vasculitis

A

U/LRTI with non life threatening organ involvement: methotrexate + steroids

32
Q

what is the management + criteria of generalised/ systemic ANCA +ive small vessel vasculitis

A

renal/ organ threatening: cyclophosphamide + steroids OR rituximab + steroids

33
Q

what is the management + criteria of refractory ANCA +ive small vessel vasculitis

A

progressive/ unresponsive to steroids: IV immunoglobins + rituximab

34
Q

what is HSP

A

ANCA -ive, IgA mediated vasculitis

35
Q

who commonly gets HSP

A

children

36
Q

what normally preceeds HSP

A

URTI, pharyngeal infection or GI infections (GAS organisms)

37
Q

what are the main symptoms of HSP (4)

A

purpura, abdo pain, arthritis, renal involvement (50%), lungs

38
Q

describe the purpura of HSP

A

purpuric (purple) rash over buttocks and lower limb (punch out legions)

39
Q

what abdo pain can come with HSP

A

colicky, bloody diarrhoea, vomiting

40
Q

what management is there for HSP

A

normally self-limiting <8 weeks, urinalysis for renal damage