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MSK > R: Vasculitis > Flashcards

R: Vasculitis Flashcards

1
Q

what is vasculitis

A

umbrella term for inflammation of vessel walls

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2
Q

what are the 2 features used to classify vasculitides?

A

size of blood vessels & presence of anti-neutrophil cytoplasmic antibodies (ANCA) in blood

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3
Q

what do we mean by large vessel?

A

aorta & its tributaries

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4
Q

pathophysiology of vasculitides?

A

dendritic cells activated releasing inflammatory cytokines > t-cells activated promoting granuloma formation & macrophage activation > mediators released causing vascular inflammation, endothelial damage etc

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5
Q

Polymyalgia reumatica and giant cell arteritis are a…

A

large vessel vasculitis

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6
Q

what are Polymyalgia reumatica and giant cell arteritis

A

granulomatous infiltrations of walls of large vessels

- GCA: arteritis of large cerebral arteries

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7
Q

s/s of Polymyalgia reumatica and giant cell arteritis

A

PMR: non-specific: fever, malaise, fatigue, hip/bum/thigh pain and stiffness that is worse in morning

GCA: headaches, tenderness of scalp, jaw claudication, visual loss, tenderness of occipital arteries

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8
Q

ix for Polymyalgia reumatica and giant cell arteritis

A

bloods: inflammatory markers
LFTs: ALP & gamma GT raised
temporal artery biopsy
MR Angio *may show thickened vessel walls)

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9
Q

mx of Polymyalgia reumatica and giant cell arteritis

A

low dose long term steroids + steroid sparing agents (MTX)

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10
Q

what is another large vessel vasculitis more common in females and asian population?

A

Takayasu’s Arteritis

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11
Q

what is a medium vessel vasculitides?

A

polyarteritis nodosa/ Kawasaki’s disease

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12
Q

epi for polyarteritis nodosa/ Kawasaki’s disease

A

PN: middle aged men

Kawasaki’s: children <5

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13
Q

aetiology of polyarteritis nodosa/ Kawasaki’s disease

A

PN: hepB?

Kawasaki’s: infective cause

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14
Q

pathophysiology of polyarteritis nodosa/ Kawasaki’s disease

A

fibrinoid necrosis of vessel walls with micro aneurysm formation, thrombosis, infarction

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15
Q

s/s of polyarteritis nodosa/ Kawasaki’s disease

A

PN: fever/wt loss

  • neuro: mononeuritis multiplex
  • renal: haematuria/ proteinuria
    cardiac: coronary arteritis

kawasakis: fever >5days, conjunctival congestion 2-4days after onset, dry/red oral cavity, cervical LN

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16
Q

ix of polyarteritis nodosa/ Kawasaki’s disease

A

PN: bloods, biopsy, angio, screen fro hepB, urinalysis
K: fever + 4/5 of other s/s + inc CRP

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17
Q

mx for polyarteritis nodosa/ Kawasaki’s disease

A

PN: corticosteroids + immunosuppression

K: immunoglobulin & aspirin

18
Q

how are small vessel vasculitides divided?

A

ANCA +Ve & ANCA -ve

19
Q

What are the anca +ve small vessel vasculitides?

A

granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis

20
Q

T/F: Wegner’s is another name for EGPA?

A

F: Wegner’s is another name for GPA

21
Q

epi for GPA & EGPA?

A

GPA: late adulthood
EGPA: early adulthood

22
Q

s/s for GPA & EGPA?

A

GPA: fever, arthralgia

  • ENT*: bloody nasal discharge, nasal discharge, otitis media
  • renal: necrotising glomerulonephritis
  • resp: cavities on x-ray, cough, pleuritic pain
  • cutaneous: non-blanching purpura rash
    neuro: CN palsies, mononeuritis multiplex

EGPA: triad of rhinitis, late onset asthma that is difficult to control, raised peripheral blood eosinophilia

23
Q

ix for GPA/EGPA?

A

GPA: biopsy*, bloods, urinalysis
- serology: c-ANCA +ve, inc PR3 antibodies

EGPA: biopsy, x-ray for pulmonary infiltrates
- serology: p-ANCA +ve

24
Q

mx for GPA/EGPA?

A

GPA: glucocorticoids & DMARDs
RGPA: corticosteroids + DMARDs

  • DMARDs= stronger given for more severe disease e.g. localised= MTX, generalised= rituximab
25
Q

golemurlonephritis is a common complication of another medium vessel vasculitis, what is this?

A

microscopic polyangiitis

26
Q

what are the 2 ANCA -ve small vessel vasculitides?

A

Henoch-Schonlein Purpura and Goodpasture’s syndrome

27
Q

what is HSP?

A

acute IgA mediated vasculitis involving GI, kidneys, joints and skin

28
Q

epi for HSP?

A

2-11 yo

29
Q

aetiology of HSP?

A

often follows an URTI, pharyngeal or GI infection

30
Q

what is the causative organism for HSP?

A

group A streptococci

31
Q

s/s of HSP?

A

purpuric skin rash on buttocks/ lower limbs, abdominal colic & bloody diarrhoea/ vomiting, glomerulonephritis

32
Q

mx for HSP?

A

self-liming (8weeks)

33
Q

what is the triad for Goodpasture’s syndrome?

A

pulmonary haemorrhage, glomerulonephritis, antibody presence to basement membrane of kidneys & lungs

34
Q

mx for GPS?

A

plasmapheresis and immunosuppression

35
Q

what is Behcet’s vasculitis?

A

chronic AI vasculitis which characteristically presents with recurrent oral and genital ulcers

36
Q

T/F: Behcet’s is a treatable condition

A

T but it is a relapsing-remitting condition

37
Q

which gene may be implicated and is a good indicator for severity in Behcet’s?

A

HLA B51

38
Q

s/s of Behcet’s affects the whole body- from lungs (pulmonary artery aneurysm) to CNS (memory impairment), but what is the classical triad?

A

oral ulcers, genital ulcers, uveitis

39
Q

what is the Ix for Behcet’s?

A

pathergy test- wheal >55mm formation with subcutaneous abrasion

40
Q

mx for Behcet’s?

A

combo of steroids & colchicine

MSK (26 decks)

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