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EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis
  1. Neil Basu1,
  2. Richard Watts2,
  3. Ingeborg Bajema3,
  4. Bo Baslund4,
  5. Thorsten Bley5,
  6. Maarten Boers6,
  7. Paul Brogan7,
  8. Len Calabrese8,
  9. Maria C Cid9,
  10. Jan Willem Cohen-Tervaert10,
  11. Luis Felipe Flores-Suarez11,
  12. Shouichi Fujimoto12,
  13. Kirsten de Groot13,
  14. Loic Guillevin14,
  15. Gulen Hatemi15,
  16. Thomas Hauser16,
  17. David Jayne17,
  18. Charles Jennette18,
  19. Cees G M Kallenberg19,
  20. Shigeto Kobayashi20,
  21. Mark A Little21,
  22. Alfred Mahr14,
  23. John McLaren22,
  24. Peter A Merkel23,
  25. Seza Ozen24,
  26. Xavier Puechal25,
  27. Niels Rasmussen4,
  28. Alan Salama26,
  29. Carlo Salvarani27,
  30. Caroline Savage21,
  31. David G I Scott28,
  32. Mårten Segelmark29,
  33. Ulrich Specks30,
  34. Cord Sunderköetter31,
  35. Kazuo Suzuki32,
  36. Vladimir Tesar33,
  37. Allan Wiik34,
  38. Hasan Yazici15,
  39. Raashid Luqmani35
  1. 1University of Aberdeen, Aberdeen, UK
  2. 2University of East Anglia, School of Medicine, Norwich, Norfolk, UK
  3. 3Leiden University Medical Center, Leiden, The Netherlands
  4. 4Rigshospitalet, Copenhagen, Denmark
  5. 5University Hospital Freiburg, Freiburg, Germany
  6. 6VU University Medical Center, Amsterdam, The Netherlands
  7. 7Institute of Child Health, London, UK
  8. 8Cleveland Clinic Foundation, Cleveland, Ohio, USA
  9. 9Hospital Clinic, University of Barcelona IDIBAPS, Barcelona, Spain
  10. 10Maastricht UMC, Maastricht, The Netherlands
  11. 11National Institute of Respiratory Disease, Mexico City, Mexico
  12. 12Miyazaki University, Miyazaki, Japan
  13. 13Klinikum Offenbach, Offenbach, Germany
  14. 14University of Paris Descartes, Paris, France
  15. 15University of Istanbul, Istanbul, Turkey
  16. 16Immunologie-Zentrum Zürich, Zurich, Switzerland
  17. 17Addenbrooke's Hospital, Cambridge, UK
  18. 18University of North Carolina, Chapel Hill, North Carolina, USA
  19. 19University Hospital Groningen, Groningen, The Netherlands
  20. 20Juntendo Koshigaya Hospital, Saitama, Japan
  21. 21Renal Institute of Birmingham, University of Birmingham, Birmingham, UK
  22. 22Whytemans Brae Hospital, Kirkcaldy, Fife, UK
  23. 23Boston University School of Medicine, Boston, Massachusetts, USA
  24. 24Hacettepe University, Ankara, Turkey
  25. 25Centre Hospitalier Le Mans, Le Mans, France
  26. 26Imperial College London, London, UK
  27. 27Arcispedale S Maria Nuova, Reggio Emilia, Italy
  28. 28Norfolk and Norwich University Hospital Trust, Norwich, Norfolk, UK
  29. 29Lund University, Lund, Sweden
  30. 30Mayo Clinc, Minnesota, Minneapolis, USA
  31. 31Universitätsklinikum Münster, Münich, Germany
  32. 32Chiba University Graduate School of Medicine, Chiba, Japan
  33. 33Charles University, Prague, Czech Republic
  34. 34Statens Serum Institut, Copenhagen, Denmark
  35. 35University of Oxford, Oxford, UK
  1. Correspondence to Raashid Luqmani, Nuffield Orthopaedic Centre, University of Oxford, Windmill Road, Oxford OX3 7LD, UK; raashid.luqmani{at}


Objectives The systemic vasculitides are multiorgan diseases where early diagnosis and treatment can significantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease definitions, classification and diagnostic criteria. Therefore, the aim of the present work was to identify deficiencies and provide contemporary points to consider for the development of future definitions and criteria in systemic vasculitis.

Methods The expert panel identified areas of concern within existing definitions/criteria. Consequently, a systematic literature review was undertaken looking to address these deficiencies and produce ‘points to consider’ in accordance with standardised European League Against Rheumatism (EULAR) operating procedures. In the absence of evidence, expert consensus was used.

Results There was unanimous consensus for re-evaluating existing definitions and developing new criteria. A total of 17 points to consider were proposed, covering 6 main areas: biopsy, laboratory testing, diagnostic radiology, nosology, definitions and research agenda. Suggestions to improve and expand current definitions were described including the incorporation of anti-neutrophil cytoplasm antibody and aetiological factors, where known. The importance of biopsy in diagnosis and exclusion of mimics was highlighted, while equally emphasising its problems. Thus, the role of alternative diagnostic tools such as MRI, ultrasound and surrogate markers were also discussed. Finally, structures to develop future criteria were considered.

Conclusions Limitations in current classification criteria and definitions for vasculitis have been identified and suggestions provided for improvement. Additionally it is proposed that, in combination with the updated evidence, these should form the basis of future attempts to develop and validate revised criteria and definitions of vasculitis.

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  • Funding EULAR Executive Secretariat, Ministerio de Cienciae Innovación, SAF 08/04328 (MCC), and the American College of Rheumatology.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Competing interest None.