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Introduction: Nomenclature and Classification

  • J. Charles JennetteEmail author
  • Ronald J. Falk
Chapter
Part of the Rare Diseases of the Immune System book series (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.

Keywords

Antineutrophil cytoplasmic antibody (ANCA) Vasculitis Microscopic polyangiitis Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis 

References

  1. 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.CrossRefGoogle Scholar
  2. 2.
    Jennette JC, Falk RJ. Pathologic classification of vasculitis. Pathol Case Rev. 2007;12:179–85.CrossRefGoogle Scholar
  3. 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. 4.
    Jennette JC, Nachman PH. ANCA glomerulonephritis and vasculitis. Clin J Am Soc Nephrol. 2017;12:1680–91.CrossRefGoogle Scholar
  5. 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.CrossRefGoogle Scholar
  6. 6.
    Willan R. On cutaneous diseases, vol. I. London: J. Johnson; 1808.Google Scholar
  7. 7.
    Schönlein JL. Allegemeine und specielle Pathologie und Therapie, vol. 2. 3rd ed. Herisau: Literatur-Comptoir; 1837. p. 48.Google Scholar
  8. 8.
    Henoch E. Uber den zusammenhang von purpura und intestinal-stoerungen. Berl Klin Wochenschur. 1868;5:517–9.Google Scholar
  9. 9.
    Henoch E. Lectures on diseases of children: a handbook for physicians and students. New York: W. Wood and Co; 1882.Google Scholar
  10. 10.
    Osler W. The visceral lesions of purpura and allied conditions. Br Med J. 1914;1:517–25.CrossRefGoogle Scholar
  11. 11.
    Goodpasture WE. The significance of certain pulmonary lesions in relation to the etiology of influenza. Am J Med Sci. 1919;158:863–70.CrossRefGoogle Scholar
  12. 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. 13.
    Dickson W. Polyarteritis acuta nodosa and periarteritis nodosa. J Pathol Bacteriol. 1908;12:31–57.CrossRefGoogle Scholar
  14. 14.
    Wohlwill F. Uber die mur mikroskopisch erkenbarre form der periarteritis nodosa. Arch Pathol Anat. 1923;246:377–411.CrossRefGoogle Scholar
  15. 15.
    Davson J, Ball M, Platt R. The kidney in periarteritis nodosa. QJM. 1948;17:175–202.PubMedGoogle Scholar
  16. 16.
    Wainwright J, Davson J. The renal appearance in the microscopic form of periarteritis nodosa. J Pathol Bacteriol. 1950;62:189–96.CrossRefGoogle Scholar
  17. 17.
    Klinger H. Grenzformen der Periarteriitis nodosa. Frankf Ztschr Pathol. 1931;42:455–80.Google Scholar
  18. 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. 19.
    Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27:277–94.PubMedPubMedCentralGoogle Scholar
  20. 20.
    Kawasaki T. MLNS showing particular skin desquamation from the finger and toe in infants. Allergy. 1967;16:178–89.PubMedGoogle Scholar
  21. 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. 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.PubMedGoogle Scholar
  23. 23.
    Godman G, Churg J. Wegener’s granulomatosis. Pathology and review of the literature. Arch Pathol Lab Med. 1954;58:533–53.Google Scholar
  24. 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.CrossRefGoogle Scholar
  25. 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.CrossRefGoogle Scholar
  26. 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.CrossRefGoogle Scholar
  27. 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.CrossRefGoogle Scholar
  28. 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.CrossRefGoogle Scholar
  29. 29.
    Tervaert JW, Elema JD, Kallenberg CG. Clinical and histopathological association of 29kD-ANCA and MPO-ANCA. APMIS Suppl. 1990;19:35.CrossRefGoogle Scholar
  30. 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.CrossRefGoogle Scholar
  31. 31.
    Yazici H. Diagnostic versus classification criteria - a continuum. Bull NYU Hosp Jt Dis. 2009;67:206–8.PubMedGoogle Scholar
  32. 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. 33.
    Jennette JC. Rapidly progressive and crescentic glomerulonephritis. Kidney Int. 2003;63:1164–72.CrossRefGoogle Scholar
  34. 34.
    Chen M, Kallenberg CG, Zhao MH. ANCA-negative pauci-immune crescentic glomerulonephritis. Nat Rev Nephrol. 2009;5:313–8.CrossRefGoogle Scholar
  35. 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.CrossRefGoogle Scholar
  36. 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.CrossRefGoogle Scholar
  37. 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.CrossRefGoogle Scholar
  38. 38.
    Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond). 2017;17:60–4.CrossRefGoogle Scholar
  39. 39.
    Scott DG, Watts RA. Epidemiology and clinical features of systemic vasculitis. Clin Exp Nephrol. 2013;17:607–10.CrossRefGoogle Scholar
  40. 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.CrossRefGoogle Scholar
  41. 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.PubMedGoogle Scholar
  42. 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.CrossRefGoogle Scholar
  43. 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.CrossRefGoogle Scholar
  44. 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

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory Medicine, School of MedicineUniversity of North Carolina at Chapel HillChapel HillUSA
  2. 2.Department of Medicine, School of MedicineUniversity of North Carolina at Chapel HillChapel HillUSA

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