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When (and how) to do a 'vasculitis screen'

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Slides created with xaringan.

Overview

  • some cases

  • clinical features of vasculitis

  • what is a 'vasculitis screen'

  • what is a 'renal screen'

  • ANCA testing

    slides at: www.kidneyfish.net/talks/

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Case 1

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23M unwell for 4 months:

[ details redacted for online slide ]

[ was a case of IgAV ]

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Chapel Hill Consensus

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Case 2

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85F unwell for 9 months:

[ details redacted for online slide ]

[ was a case of AAV with ENT, nerve, skin and kidney disase ]

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Questions

  • what is the differential diagnosis?

  • what tests would you do?

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Questions

  • what is the differential diagnosis?

  • what tests would you do?


  • if she is ANCA +ve, what is the probability that this is vasculitis?

  • if she is ANCA -ve, what is the probability that this is vasculitis?

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ANCA seropositivity in 1 in 300 blood donors (c.f. 1 in 6 for ANA).

Pre-test probability if sinus and GN = 0.3.

ANCA vasculitis

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Indications for ANCA testing

Hunter et al. (2020)

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ANCA false positives

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Investigations in suspected vasculitis

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'Renal screen'

Consider:

  • look closely at routine tests / previous trends
  • PR3, MPO, GBM
  • C3, C4
  • ANA, ENA, dsDNA
  • paraprotein screen, immunoglobulins
  • HepB, HepC, HIV
  • CK
  • blood film, haemolysis screen
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'Renal screen'

Trak options:

  • vasculitis screen = ANA, PR3, MPO, C3, C4
  • connective tissue screen = ANA, DNA, ENA, C3, C4, CCP
  • autoimmune profile = ANA, DNAIF, GPC, AMA, SMA, TPO
  • glomerulonephritis = vasculitis screen + GBM + ENA screen

(ENA = Ro, La, SmDP, U1RNP, Scl70S, Jo1)

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Take-home messages

  • there is no catch-all 'renal screen'

  • test for vasculitis if there is a reasonably high pre-test probability

  • send PR3, MPO (+/- GBM) and phone immunology labs

  • ANCA not 100% sensitive or specific

  • indications include: suspected GN, ENT disease, alveolar haemorrhage, pulmonary nodules, scleritis, neuropathy, rash with systemic features

  • if suspecting vasculitis (or intrinsic renal disease) then ask renal

    slides at: www.kidneyfish.net/talks/

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Overview

  • some cases

  • clinical features of vasculitis

  • what is a 'vasculitis screen'

  • what is a 'renal screen'

  • ANCA testing

    slides at: www.kidneyfish.net/talks/

2 / 23
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