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Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis pp 3–17Cite as

Introduction: Nomenclature and Classification

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Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Nomenclature, classification, and diagnosis of vasculitis are difficult because of the broad spectrum of types and locations of vessels affected, multiple patterns of injury, diverse known etiologies and pathogenic mechanisms, absence of known etiologies and pathogenic mechanisms in some forms of vasculitis, and the myriad overlapping and nonspecific signs and symptoms caused by vasculitides. The goals of nomenclature, classification, and diagnostic systems are to enable effective communication among biomedical investigators and healthcare providers, guide clinical and basic research on well-defined cohorts (classes) of patients, and, most importantly, facilitate diagnosis and effective treatment of individual patients. This chapter reviews the historical basis for vasculitis nomenclature, classification, and diagnosis and the use of pathologic, clinical, and serologic data for classification and diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Physicians and scientists have made remarkable advances in the classification and diagnosis of vasculitides since the seminal observations in the 1800s; however, validated and widely applied classification criteria and diagnostic criteria that are sufficiently accurate and precise for clinical research and patient care, respectively, remain elusive. Even after well-validated criteria are established, they must be under constant scrutiny and adjusted as new knowledge emerges and new diagnostic modalities are introduced.

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References

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.

    Article  CAS  Google Scholar 

  2. Jennette JC, Falk RJ. Pathologic classification of vasculitis. Pathol Case Rev. 2007;12:179–85.

    Article  Google Scholar 

  3. Jennette JC, Thomas DB. Pauci-immune and antineutrophil cytoplasmic autoantibody glomerulonephritis and vasculitis. In: Jennette JC, Olson JL, Silva FG, D’Agati V, editors. Heptinstall’s pathology of the kidney. 7th ed. Philadelphia: Wolters Kluwer; 2015, Chapter 16. p. 685–714.

    Google Scholar 

  4. Jennette JC, Nachman PH. ANCA glomerulonephritis and vasculitis. Clin J Am Soc Nephrol. 2017;12:1680–91.

    Article  Google Scholar 

  5. Cornec D, Cornec-Le Gall E, Fervenza FC, Specks U. ANCA-associated vasculitis - clinical utility of using ANCA specificity to classify patients. Nat Rev Rheumatol. 2016;12:570–9.

    Article  CAS  Google Scholar 

  6. Willan R. On cutaneous diseases, vol. I. London: J. Johnson; 1808.

    Google Scholar 

  7. Schönlein JL. Allegemeine und specielle Pathologie und Therapie, vol. 2. 3rd ed. Herisau: Literatur-Comptoir; 1837. p. 48.

    Google Scholar 

  8. Henoch E. Uber den zusammenhang von purpura und intestinal-stoerungen. Berl Klin Wochenschur. 1868;5:517–9.

    Google Scholar 

  9. Henoch E. Lectures on diseases of children: a handbook for physicians and students. New York: W. Wood and Co; 1882.

    Google Scholar 

  10. Osler W. The visceral lesions of purpura and allied conditions. Br Med J. 1914;1:517–25.

    Article  CAS  Google Scholar 

  11. Goodpasture WE. The significance of certain pulmonary lesions in relation to the etiology of influenza. Am J Med Sci. 1919;158:863–70.

    Article  Google Scholar 

  12. Kussmaul A, Maier R. Über eine bisher nicht beschreibene eigenthümliche Arterienerkrankung (Periarteriitis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med. 1866;1:484–518.

    Google Scholar 

  13. Dickson W. Polyarteritis acuta nodosa and periarteritis nodosa. J Pathol Bacteriol. 1908;12:31–57.

    Article  Google Scholar 

  14. Wohlwill F. Uber die mur mikroskopisch erkenbarre form der periarteritis nodosa. Arch Pathol Anat. 1923;246:377–411.

    Article  Google Scholar 

  15. Davson J, Ball M, Platt R. The kidney in periarteritis nodosa. QJM. 1948;17:175–202.

    CAS  PubMed  Google Scholar 

  16. Wainwright J, Davson J. The renal appearance in the microscopic form of periarteritis nodosa. J Pathol Bacteriol. 1950;62:189–96.

    Article  CAS  Google Scholar 

  17. Klinger H. Grenzformen der Periarteriitis nodosa. Frankf Ztschr Pathol. 1931;42:455–80.

    Google Scholar 

  18. Wegener F. Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems unter den Nieren. Beitr Pathol Anat. 1939;102:36–68.

    Google Scholar 

  19. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27:277–94.

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Kawasaki T. MLNS showing particular skin desquamation from the finger and toe in infants. Allergy. 1967;16:178–89.

    CAS  PubMed  Google Scholar 

  21. Tanaka N, Naoe S, Kawasaki T. Pathological study on autopsy cases of mucocutaneous lymph node syndrome. J Jpn Red Cross Central Hosp. 1971;2:85–94.

    Google Scholar 

  22. Karadag O, Jayne DJ. Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda. Clin Exp Rheumatol. 2018;36 Suppl 111(2):135–42.

    PubMed  Google Scholar 

  23. Godman G, Churg J. Wegener’s granulomatosis. Pathology and review of the literature. Arch Pathol Lab Med. 1954;58:533–53.

    CAS  Google Scholar 

  24. Guillevin L1, Lhote F, Amouroux J, Gherardi R, Callard P, Casassus P. Antineutrophil cytoplasmic antibodies, abnormal angiograms and pathological findings in polyarteritis nodosa and Churg-Strauss syndrome: indications for the classification of vasculitides of the polyarteritis Nodosa group. Br J Rheumatol. 1996;35:958–64.

    Article  CAS  Google Scholar 

  25. Faille-Kuyber EH, Kater L, Kooiker CJ, Dorhout Mees EJ. IgA-deposits in cutaneous blood-vessel walls and mesangium in Henoch-Schönlein syndrome. Lancet. 1973;1:892–3.

    Article  CAS  Google Scholar 

  26. Meltzer M, Franklin EC, Elias K, McCluskey RT, Cooper N. Cryoglobulinemia-a clinical and laboratory study. II. Cryoglobulins with rheumatoid factor activity. Am J Med. 1966;40:837–56.

    Article  CAS  Google Scholar 

  27. van der Woude FJ, Rasmussen N, Lobatto S, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet. 1985;1:425–9.

    Article  Google Scholar 

  28. Falk RJ, Jennette JC. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med. 1988;318:1651–7.

    Article  CAS  Google Scholar 

  29. Tervaert JW, Elema JD, Kallenberg CG. Clinical and histopathological association of 29kD-ANCA and MPO-ANCA. APMIS Suppl. 1990;19:35.

    Article  CAS  Google Scholar 

  30. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides: the proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–92.

    Article  CAS  Google Scholar 

  31. Yazici H. Diagnostic versus classification criteria - a continuum. Bull NYU Hosp Jt Dis. 2009;67:206–8.

    PubMed  Google Scholar 

  32. Smith HW. Renal physiology. In: Fishman AP, Richards DW, editors. Circulation of the blood: men and ideas. New York: Springer; 1982, Chapter 9. p. 581.

    Google Scholar 

  33. Jennette JC. Rapidly progressive and crescentic glomerulonephritis. Kidney Int. 2003;63:1164–72.

    Article  Google Scholar 

  34. Chen M, Kallenberg CG, Zhao MH. ANCA-negative pauci-immune crescentic glomerulonephritis. Nat Rev Nephrol. 2009;5:313–8.

    Article  CAS  Google Scholar 

  35. Craven A, Robson J, Ponte C, et al. ACR/EULAR-endorsed study to develop diagnostic and classification criteria for vasculitis (DCVAS). Clin Exp Nephrol. 2013;17:619–21.

    Article  CAS  Google Scholar 

  36. Pearce FA, Craven A, Merkel PA, et al. Global ethnic and geographic differences in the clinical presentations of anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology. 2017;56:1962–9.

    Article  CAS  Google Scholar 

  37. Lionaki S, Blyth ER, Hogan SL, 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. 2012;64:3452–62.

    Article  CAS  Google Scholar 

  38. Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond). 2017;17:60–4.

    Article  Google Scholar 

  39. Scott DG, Watts RA. Epidemiology and clinical features of systemic vasculitis. Clin Exp Nephrol. 2013;17:607–10.

    Article  Google Scholar 

  40. Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52:2926–35.

    Article  CAS  Google Scholar 

  41. Sokolowska BM, Szczeklik WK, Wludarczyk AA, et al. ANCA-positive and ANCA-negative phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA): outcome and long-term follow-up of 50 patients from a single Polish center. Clin Exp Rheumatol. 2014;32:S41–7.

    PubMed  Google Scholar 

  42. Cottin V, Bel E, Bottero P, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): a study of 157 patients by the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev. 2017;16:1–9.

    Article  CAS  Google Scholar 

  43. Watts R, Lane S, Hanslik T, Hauser T, 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. 2007;66:222–7.

    Article  Google Scholar 

  44. Fries JF, Hunder GG, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. 1990;33:1135–6.

    Google Scholar 

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

    J. Charles Jennette

  2. Department of Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

    Ronald J. Falk

Authors
  1. J. Charles Jennette
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  2. Ronald J. Falk
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Correspondence to J. Charles Jennette .

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Editors and Affiliations

  1. Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Monza e Brianza, Italy

    Renato Alberto Sinico

  2. Department of Medicine, Paris Descartes University Hospital Cochin, Paris, France

    Loïc Guillevin

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Jennette, J.C., Falk, R.J. (2020). Introduction: Nomenclature and Classification. In: Sinico, R., Guillevin, L. (eds) Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-02239-6_1

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  • DOI: https://doi.org/10.1007/978-3-030-02239-6_1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-02238-9

  • Online ISBN: 978-3-030-02239-6

  • eBook Packages: MedicineMedicine (R0)

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