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Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies
  1. Richard Watts1,
  2. Suzanne Lane2,
  3. Thomas Hanslik3,
  4. Thomas Hauser4,
  5. Bernhard Hellmich5,
  6. Wenche Koldingsnes6,
  7. Alfred Mahr7,
  8. Mårten Segelmark8,
  9. Jan W Cohen-Tervaert9,
  10. David Scott10
  1. 1School of Medicine, Health Policy and Practice, University of East Anglia, Norwich, UK
  2. 2Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK
  3. 3AP-HP, Ambroise Paré Hospital, Department of Internal Medicine, University of Versailles Saint-Quentin-en-Yvelines, Yvelines, France
  4. 4Division of Clinical Immunology, University Hospital, Zurich, Switzerland
  5. 5University of Lubeck, Lubeck, Germany
  6. 6Department of Rheumatology, University Hospital Northern Norway, Tromsφ, Norway
  7. 7Department of Internal Medicine, Hôpital Cochin Paris, Paris, France
  8. 8Department of Nephrology, University Hospital Lund, Lund, Sweden
  9. 9Department of Clinical and Experimental Immunology, University Hospital Maastricht, Maastricht, The Netherlands
  10. 10Department of Rheumatology, Norfolk and Norwich University Trust, Norwich, UK
  1. Correspondence to:
    Dr R Watts
    Department of Rheumatology, Ipswich Hospital NHS Trust, Heath Road, Ipswich IP4 5PD, UK; richard.watts{at}


Background: The classification of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and polyarteritis nodosa (PAN) for epidemiology studies is confusing. The existing schemes such as American College of Rheumatology (ACR) criteria, Chapel Hill Consensus Conference (CHCC) definitions and Lanham criteria produce overlapping and conflicting classifications, making it difficult to compare incidence figures.

Aim: To develop a consensus method of using these criteria and definitions for epidemiological studies to permit comparison without confounding by classification.

Methods: A stepwise algorithm was developed by consensus between a group of doctors interested in the epidemiology of vasculitis. The aim was to categorise patients with Wegener’s granulomatosis, microscopic polyangiitis (MPA), Churg–Strauss syndrome (CSS) and PAN into single clinically relevant categories. The ACR and Lanham criteria for CSS, and ACR criteria for Wegener’s granulomatosis were applied first, as these were considered to be the most specific. Surrogate markers for Wegener’s granulomatosis were included to distinguish Wegener’s granulomatosis from MPA. MPA was classified using the CHCC definition and surrogate markers for renal vasculitis. Finally, PAN was classified using the CHCC definition. The algorithm was validated by application to 20 cases from each centre and 99 from a single centre, followed by a paper case exercise.

Results: A four-step algorithm was devised. It successfully categorises patients into a single classification. There was good correlation between observers in the paper case exercise (91.5%; unweighted κ = 0.886).

Conclusion: The algorithm achieves its aim of reliably classifying patients into a single category. The use of the algorithm in epidemiology studies should permit comparison between geographical areas.

  • AAV, ANCA-associated vasculitis
  • ACR, American College of Rheumatology
  • ANCA, antineutrophil cytoplasmic antibody
  • CHCC, Chapel Hill Consensus Conference
  • cPAN, classic polyarteritis nodosa
  • CSS, Churg–Strauss syndrome
  • HSP, Henoch Schonlein purpura
  • IIF, indirect immunofluorescence
  • MPA, microscopic polyangiitis
  • MPO, myeloperoxidase
  • PAN, polyarteritis nodosa

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