Abstract
A nomenclature system provides names and definitions for diseases, and provides the framework for establishing classification criteria for groups of patients and diagnostic criteria for individual patients. The International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC) provides standardized names and definitions for different classes of vasculitis, but does not provide validated criteria for classifying cohorts of patients into these classes, or for diagnosing (classifying) an individual patient. The CHCC nomenclature and definitions are useful for communication among health care providers, understanding the medical literature, guiding development of classification and diagnostic criteria, and facilitating research on cohorts of patients with vasculitis. Names and definitions evolve more slowly than classification and diagnostic criteria because the latter must change as new diagnostic technologies and clinical laboratory testing are available. For example, the discovery of anti-neutrophil cytoplasmic autoantibodies (ANCA) added a new criterion for classifying vasculitis. The most robust ongoing effort to develop classification and diagnostic criteria for vasculitis is by the Diagnostic and Classification Criteria for Vasculitis (DCVAS) study group. Once data are collected from large vasculitis patient cohorts, identifying the most clinically and biologically relevant classes, and the most accurate and precise diagnostic criteria, may require the application of supervised and unsupervised machine learning algorithms. It will be interesting to see how machine generated vasculitis classes agree (or not) with the CHCC classes that were devised by mere mortals.
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References
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CGM, McCluskey RT, Sinico RA, Rees AJ, van Es LA, Waldherr R, Wiik A (1994) Nomenclature of systemic vasculitides: the proposal of an international consensus conference. Arthritis Rheum 37:187–192
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CGM, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DGI, Specks U, Stone JH, Takahashi K, Watts RA (2013) 2012 revised international Chapel Hill consensus conference nomenclature of Vasculitides. Arthritis Rheum 65:1–11
Jennette JC, Falk RJ (1997) Small vessel vasculitis. N Engl J Med 337:1512–1523
Jennette JC, Falk RJ (2007) Pathologic classification of vasculitis. Pathol Case Rev 12:179–185
Falk RJ, Gross WL, Guillevin L, Hoffman G, Jayne DRW, Jennette JC et al (2011) “Granulomatosis with polyangiitis (Wegener’s)”: an alternative name for “Wegener’s granulomatosis”. A joint proposal of the American College of Rheumatology, the American Society of Nephrology, and the European League Against Rheumatism. Ann Rheum Dis 70:704. J Am Soc Nephrol 22:587–588. Arthritis Rheum 63:863–864
Lionaki S, Blyth ER, Hogan SL, Hu Y, Senior JBA, Jennette CE, Nachman PH, Jennette JC, Falk RJ (2012) 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 64:3452–3462
Mahr A, Katsahian S, Varet H, Guillevin L, Hagen EC, Höglund P, Merkel PA, Pagnoux C, Rasmussen N, Westman K, Jayne DR, French Vasculitis Study Group (FVSG) and the European Vasculitis Society (EUVAS) (2013) Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: a cluster analysis. Ann Rheum Dis 72:1003–1010
Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DR, Baslund B, Brenchley P, Bruchfeld A, Chaudhry AN, Cohen Tervaert JW, Deloukas P, Feighery C, Gross WL, Guillevin L, Gunnarsson I, Harper L, Hrušková Z, Little MA, Martorana D, Neumann T, Ohlsson S, Padmanabhan S, Pusey CD, Salama AD, Sanders JS, Savage CO, Segelmark M, Stegeman CA, Tesař V, Vaglio A, Wieczorek S, Wilde B, Zwerina J, Rees AJ, Clayton DG, Smith KG (2012) Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 367:214–223
Jennette JC, Falk RJ (2014) Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease. Nat Rev Rheumatol 10:463–473
Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E et al (2006) Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 47:770–779
Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA (2011) The pathophysiology of IgA nephropathy. J Am Soc Nephrol 22:1795–1803
Kallenberg CG (2008) Anti-C1q autoantibodies. Autoimmun Rev 7:612–615
Craven A, Robson J, Ponte C, Grayson PC, Suppiah R, Judge A, Watts R, Merkel PA, Luqmani RA (2013) ACR/EULAR-endorsed study to develop diagnostic and classification criteria for vasculitis (DCVAS). Clin Exp Nephrol 17:619–621
Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D (2007) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66:222–227
Fries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Colabrese LH (1990) The American College of Rheumatology 1990 criteria for the classification of vasculitis: summary. Arthritis Rheum 33:1135–1136
Lanham JG, Elkon KB, Pusey CD, Hughes GR (1984) Systemic vasculitis in asthma and eosinophilia: a clinical approach to the Churg Strauss syndrome. Medicine (Baltimore) 63:65–81
Kamali S, Artim-Esen B, Erer B, Ozdener L, Gul A, Ocal L, Aral O, Inanc M (2012) Re-evaluation of 129 patients with systemic necrotizing vasculitides by using classification algorithm according to consensus methodology. Clin Rheumatol 31:325–328
Liu LJ, Chen M, Yu F, Zhao MH, Wang HY (2008) Evaluation of a new algorithm in classification of systemic vasculitis. Rheumatology (Oxford) 47:708–712
Abdulkader R, Lane SE, Scott DG, Watts RA (2013) Classification of vasculitis: EMA classification using CHCC 2012 definitions. Ann Rheum Dis 72:1888
Uribe AG, Huber AM, Kim S, O'Neil KM, Wahezi DM, Abramson L, Baszis K, Benseler SM, Bowyer SL, Campillo S, Chira P, Hersh AO, Higgins GC, Eberhard A, Ede K, Imundo LF, Jung L, Kingsbury DJ, Klein-Gitelman M, Lawson EF, Li SC, Lovell DJ, Mason T, McCurdy D, Muscal E, Nassi L, Rabinovich E, Reiff A, Rosenkranz M, Schikler KN, Singer NG, Spalding S, Stevens AM, Cabral DA, A Registry for Children with Vasculitis e-entry (ARChiVe) Network (2012) Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis. J Rheumatol 39:1687–1697
Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, Kawasaki T, Lindsley C, Petty RE, Prieur AM, Ravelli A, Woo P (2006) EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 65:936–941
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (PRINTO) (2010) 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 69:798–806
Ponte C, Grayson PC, Suppiah R, Robson J, Anthea C, Judge A, Merkel PA, Watts RA, Luqmani RA (2014) Development of the classification criteria for giant cell arteritis in the diagnostic and classification criteria for vasulitis study: a pilot study using a panel review methodology. Ann Rheum Dis 73(Suppl 2):555 (abstract)
Unizony SH, Dasgupta B, Fisheleva E, Rowell L, Schett G, Spiera R, Zwerina J, Harari O, Stone JH (2013) Design of the tocilizumab in giant cell arteritis trial. Int J Rheumatol 2013, 912562
Linder R, Orth I, Hagen EC, van der Woude FJ, Schmitt WH (2011) Differentiation between Wegener’s granulomatosis and microscopic polyangiitis by an artificial neural network and by traditional methods. J Rheumatol 38:1039–1047
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Jennette, J.C., Falk, R.J., Alba, M.A. (2016). Nomenclature of Vasculitides: 2012 Revised International Chapel Hill Consensus Conference. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_3
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