Vasculitis Flashcards

1
Q

What are the different categories of vasculitis?

A
  • immune complex small vessel vasculitis
  • ANCA-associated small vessel vasculitis
  • medium vessel vasculitis
  • large vessel vasculitis
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2
Q

What types of vasculitis is immune complex small vessel vasculitis?

A
  • cryoglobulinemic vasculitis
  • IgA vasculitis
  • hypocomplementemic urticarial vasculitis
  • anti-GBM disease
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3
Q

What type of vasculitis is in ANCA-associated small vessel vasculitis?

A
  • microscopic polyangiitis
  • granulomatosis with polyangiitis
  • eosinophilic granulomatosis with polyangiitis
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4
Q

What vasculitis is in medium vessel vasculitis/

A
  • polyarteritis nodosa
  • Kawasaki disease
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5
Q

What vasculitis is in large vessel vasculitis?

A
  • takayasu arteritis
  • giant cell arteritis
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6
Q

What sign might you see on examination in giant cell arteritis?

A

thickened, non-pulsatile temporal artery

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7
Q

Define giant cell arteritis

A

systemic vasculitis that affects the aorta and its major branches

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8
Q

Describe a typical clinical presentation of giant cell arteritis

A
  • headache (temporal with pain on palpation, subacute onset, constant with little relief from analgesics)
  • visual symptoms
  • jaw claudication (pain when eating/talking)
  • polymyalgia reumatica symptoms (shoulder/pelvic girdle pain)
  • constitutional symptoms
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9
Q

What are the complications of giant cell arteritis?

A
  • visual loss (irreversible loss, acute ischaemic neuropathy, sudden painless loss - can be preceded by amaurosis fugal)
  • large vessel vasculitis (stenosis and aneurysm - stroke risk)
  • CVA (obstruction/occlusion of internal carotid a./vertebral a.
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10
Q

What investigations would you want to do in giant cell arteritis?

A
  • temporal artery biopsy (gold standard)
  • temporal artery US
  • MRI
  • PET CT (good to check if large vessel involvement - aneurysm)
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11
Q

How would you medically treat giant cell arteritis?

A
  • prednisolone 1mg/kg/day (discontinue by 12-18months)
    • aspirin 75mg (if risk for stroke)
  • methotrexate/ mycophenolate mofetil/ tocilizumab (steroid-sparing therapy) for relapses
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12
Q

What are the causes of cutaneous vasculitis?

A
  • idiopathic
  • drugs
  • infection (HCV, HBV, gonococcus, meningococcus, HIV)
  • secondary RA/CTD/PBC/UC
  • malignancy (esp lymphoma)
  • manifestation of small/medium ANCA vasculitis
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13
Q

What important tests should you do if you suspect vasculitis?

A
  • CRP/ESR
  • urinalysis (kidney function)
  • FBC, LFTs, Us and Es
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14
Q

Describe the signs of Henoch Schonlein purpura

A
  • purpuric rash on buttocks and thighs
  • urticarial rash, petechiae, ecchymoses, ulcers
  • arthralgia/arthritis (lower limb)
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15
Q

What are the complications of Henoch Schonlein purpura?

A
  • GI: pain, bleeding, diarrhoea, intussusception (rare)
  • renal: IgA nephropathy
  • urinary: orchitis
  • CNS (rare)
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16
Q

What is the management and prognosis for Henoch Schonlein purpura?

A
  • often no treatment
  • corticosteroids for certain complications (testicular torsion, GI, arthritis)
  • self-limiting with small minority relapsing within 12 months
17
Q

Describe granulomatosis with polyangiitis (GPA)

A

granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract and often a pauci-immune glomerulonephritis (no immune deposit in glomeruli)

18
Q

What is the classical triad of symptoms and signs of GPA?

A
  • upper airway symptoms: rhinitis, chronic sinusitis, chronic otitis media, saddle nose deformity, nasal septal perforation
  • renal symptoms: pauci-immune glomerulonephritis
  • lower respiratory symptoms: parenchymal nodules and cavitation, alveolar haemorrhage (haemoptysis)
19
Q

What factors would increase your clinical suspicion of vasculitis?

A
  • classical presentation
  • constitutional symptoms
  • multi-system disease
  • repeat GP/hospital visits
  • disease ‘not behaving’ as it should
20
Q

Describe the immunology of ANCA vasculitis

A
  • autoantibodies against cytoplasmic constituents of neutrophils and monocytes
  • cANCA with PR3 (neutrophils) very suggestive of GPA
  • pANCA with strong MPO suggestive of MPA (or EGPA)
  • positive not always indicative of ANCA
21
Q

How useful is ANCA detection?

A
  • not helpful as diagnostic tool (not all positive ANCE = ANCA vasculitis)
  • useful prognostic information (increased ANCA = increased chance of relapse)
  • can monitor for for early signs of relapse)
22
Q

What is the treatment for vasculitis?

A
  1. remission induction (prednisolone + cyclophosphamide/rituximab or methotrexate/mycophenolate - milder drugs)
    - switch off vasculitis activity
    - higher dose = higher toxicity
    - 3-6m
  2. remission maintenance (azathioprine/ methotrexate/ rituximab)
    - prevent relapse
    - lower drug toxicities
    - more prolonged therapy
23
Q

What are the risks of cyclophosphamide treatment?

A
  • cytopenias
  • malignancy
  • infertility
  • rituximab is safer and no risk of infertility