Skip to main content

Advertisement

SpringerLink
Log in

Classification of ANCA-Associated Vasculitis

  • VASCULITIS (LR ESPINOZA, SECTION EDITOR)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Classification of the ANCA-associated vasculitides remains controversial. Existing systems, developed by the American College of Rheumatology (ACR) in 1990, the Chapel Hill Consensus Conference (CHCC) in 1994 and updated in 2012, and the European Medicines Agency algorithm, all have deficiencies, especially when applied to unselected patients. The ACR system did not include ANCA or microscopic polyangiitis, and the CHCC (1994) included MPA but not ANCA (this was rectified in the 2012 revision). These systems were developed as classification criteria and not as diagnostic criteria. There are currently no validated diagnostic criteria for AAV. The Diagnostic and Classification Criteria for Vasculitis (DCVAS) study is a global study with the objective of developing and validating diagnostic criteria.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. •• Jennette J, Falk R, Bacon P, Basu N, Cid M, Ferrario F, et al. 2012 Revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum [Internet]. 2013 Oct 8 [cited 2012 Nov 1];65:1–11. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23045170. This paper describes the revised Chapel Hill Nomenclature for vasculitis, with an expanded nomenclature and definitions for all commonly encountered forms of vasculitis.

  2. Fries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum [Internet]. 1990 Aug [cited 2013 Sep 24];33(8):1135–6. Available from: http://www.ncbi.nlm.nih.gov/pubmed/2202312.

  3. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum [Internet]. 1994 Feb [cited 2012 Dec 19];37(2):187–92. Available from: http://www.ncbi.nlm.nih.gov/pubmed/8129773.

  4. Van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet [Internet]. 1985 Feb 23 [cited 2012 Dec 17];1(8426):425–9. Available from: http://www.ncbi.nlm.nih.gov/pubmed/2857806.

  5. Watts R A, Scott DGI. ANCA vasculitis: to lump or split?: Why we should study MPA and GPA separately. Rheumatology (Oxford) [Internet]. 2012 Aug 25 [cited 2012 Sep 4];3–5. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22923757.

  6. Watts RA, Mooney J, Skinner J, Scott DGI, Macgregor AJ. The contrasting epidemiology of granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis. Rheumatology [Internet]. 2012 May [cited 2013 Mar 8];51(5):926–31. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22258386.

  7. Stegeman C, Tervaert J, Sluiter W, Manson W, de Jong P, Kallenberg C. Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med. 1994;120:12–7.

    Article  PubMed  CAS  Google Scholar 

  8. Chen M, Kallenberg CGM. The environment, geoepidemiology and ANCA-associated vasculitides. Autoimmun Rev [Internet]. 2010 Mar [cited 2013 Jan 30];9(5):A293–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/19892038.

  9. •• Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DRW, et al. Genetically distinct subsets within ANCA-Associated vasculitis. N Engl J Med [Internet]. 2012 Jul 19 [cited 2012 Jul 19];367(3):214–23. Available from: http://www.nejm.org/doi/abs/10.1056/NEJMoa1108735. This paper described the results of the first genome wide association study in ANCA-vasculitis demonstrating the different genetic background to GPA and MPA.

  10. Walsh M, Flossmann O, Berden A, Westman K, Höglund P, Stegeman C, et al. Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum [Internet]. 2012 Mar [cited 2012 Oct 31];64(2):542–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/21953279.

  11. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum [Internet]. 1990 Aug [cited 2013 Sep 24];33(8):1101–7. Available from: http://www.ncbi.nlm.nih.gov/pubmed/2202308.

  12. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum [Internet]. 1990 Aug [cited 2013 Sep 24];33(8):1094–100. Available from: http://www.ncbi.nlm.nih.gov/pubmed/2202307.

  13. Watts RA, Jolliffe VA, Carruthers DM, Lockwood M, Scott DG. Effect of classification on the incidence of polyarteritis nodosa and microscopic polyangiitis. Arthritis Rheum [Internet]. 1996 Jul [cited 2013 Sep 24];39(7):1208–12. Available from: http://www.ncbi.nlm.nih.gov/pubmed/8670332.

  14. Sørensen SF, Slot O, Tvede N, Petersen J. A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature. Ann Rheum Dis [Internet]. 2000 Jun [cited 2013 Sep 24];59(6):478–82. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1753166&tool=pmcentrez&rendertype=abstract.

    Google Scholar 

  15. Lane SE, Watts RA, Barker THW, Scott DGI. Evaluation of the Sørensen diagnostic criteria in the classification of systemic vasculitis. Rheumatology (Oxford) [Internet]. 2002 Oct [cited 2013 Sep 24];41(10):1138–41. Available from: http://www.ncbi.nlm.nih.gov/pubmed/12364633.

  16. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis [Internet]. 2007 Feb [cited 2012 Jul 28];66(2):222–7. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1798520&tool=pmcentrez&rendertype=abstract.

  17. Liu L-J, Chen M, Yu F, Zhao M-H, Wang H-Y. Evaluation of a new algorithm in classification of systemic vasculitis. Rheumatology (Oxford) [Internet]. 2008 May [cited 2012 Dec 7];47(5):708–12. Available from: http://www.ncbi.nlm.nih.gov/pubmed/18390585.

  18. Kamali S, Artim-Esen B, Erer B, Ozdener L, Gul A, Ocal L, et al. Re-evaluation of 129 patients with systemic necrotizing vasculitides by using classification algorithm according to consensus methodology. Clin Rheumatol [Internet]. 2012 Feb [cited 2013 Jan 18];31(2):325–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/21814755.

  19. Abdulkader R, Lane SE, Scott DGI, Watts RA. Classification of vasculitis: EMA classification using CHCC 2012 definitions. Ann Rheum Dis [Internet]. 2013 Jun 1 [cited 2013 Sep 18]; Available from: http://www.ncbi.nlm.nih.gov/pubmed/23727632.

  20. Mahr A, Katsahian S, Varet H, Guillevin L, Hagen EC, Höglund P, et al. Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: a cluster analysis. Ann Rheum Dis [Internet]. 2012 Sep 8 [cited 2012 Sep 21];1–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22962314.

  21. Lionaki S, Blyth ER, Hogan SL, Hu Y, Senior BA, Jennette CE, et al. Classification of antineutrophil cytoplasmic autoantibody vasculitides: the role of antineutrophil cytoplasmic autoantibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis. Arthritis Rheum [Internet]. 2012 Oct [cited 2012 Oct 3];64(10):3452–62. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23023777.

  22. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis [Internet]. 2010 May [cited 2013 Sep 24];69(5):798–806. Available from: http://www.ncbi.nlm.nih.gov/pubmed/20413568.

  23. Uribe AG, Huber AM, Kim S, O’Neil KM, Wahezi DM, Abramson L, et al. Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis. J Rheumatol [Internet]. 2012 Aug [cited 2013 Jan 18];39(8):1687–97. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22589257.

  24. • Craven A, Robson J, Ponte C, Grayson PC, Suppiah R, Judge A, et al. ACR/EULAR-endorsed study to develop Diagnostic and Classification Criteria for Vasculitis (DCVAS). Clin Exp Nephrol [Internet]. 2013 Aug 31 [cited 2013 Sep 16]; Available from: http://www.ncbi.nlm.nih.gov/pubmed/23996327. This paper describes the global effort to develop new classification criteria for the vasculitides.

  25. Singh J, Solomon D, Dougados M, Felson D, Hawker G, Katz P, et al. Development of classification and response criteria for rheumatic diseases. Arthritis Rheum. 2006;55(3):348–52.

    Article  PubMed  Google Scholar 

  26. Watts RA, Suppiah R, Merkel P, Luqmani R. Systemic vasculitis: is it time to reclassify? Rheumatology (Oxford). 2011;50(4):643–5.

    Article  Google Scholar 

Download references

Compliance with Ethics Guidelines

Conflict of Interest

Irfan Khan and Richard A. Watts declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

  1. Department of Rheumatology, Ipswich Hospital NHS Trust, Heath Road, Ipswich, IP4 5PD, UK

    Irfan Khan & Richard A. Watts

  2. Norwich Medical School, University of East Anglia, Norwich, NR4 7TJ, UK

    Richard A. Watts

Authors
  1. Irfan Khan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Richard A. Watts
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Richard A. Watts.

Additional information

This article is part of the Topical Collection on Vasculitis

Rights and permissions

Reprints and permissions

About this article

Cite this article

Khan, I., Watts, R.A. Classification of ANCA-Associated Vasculitis. Curr Rheumatol Rep 15, 383 (2013). https://doi.org/10.1007/s11926-013-0383-6

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11926-013-0383-6

Keywords

Search

Navigation