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Do vasculitis categorization systems really matter?

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Abstract

The diagnosis of systemic vasculitides is challenging for many reasons. The etiology and pathogenesis of most vasculitides are unknown or incompletely known. Vasculitides have protean and overlapping clinical and pathologic features. There are conflicting if not contradictory approaches to diagnostic categorization. In spite of these challenges, precise diagnostic categorization is essential for appropriate treatment. This overview reviews the history behind the modern approach to diagnosis of selected vasculitides, including giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, Kawasaki disease, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Evidence is provided that the categorization for systemic vasculitis really does matter.

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References and Recommended Reading

  1. Papper,S: Clinical Nephrology, edn 2. Boston: Little, Brown, & Co.; 1978:171.

    Google Scholar 

  2. Saxe,JG: The Poetical Works of John Godfrey Saxe. Boston: Houghton, Mifflin and Co.; 1892:111–112.

    Google Scholar 

  3. Jennette,JC, Falk,RJ, Andrassy,K, et al.:Nomenclature of systemic vasculitides: the proposal of an international consensus conference. Arthritis Rheum 1994, 37:187-192. 4. Savory WS: Case of a young woman in whom the main arteries of both upper extremities and of the left side of the neck were throughout completely obliterated. Med Chir Trans Lond 1856, 39:205–219.

    Google Scholar 

  4. Takayasu,M: Case with unusual changes of the central vessels in the retina. Acta Soc Ophthalmol Jpn 1908, 12:554–563.

    Google Scholar 

  5. Hutchinson,J: Diseases of the arteries: on a peculiar form of thrombotic arteries of the aged which is sometimes productive of gangrene. Arch Surg [London] 1890, 1:323.

    Google Scholar 

  6. Rokitansky,K: Ueber einige der wichtigsten Krankheiten der Arterien. Denkschriften der Kais Akad der Wissensch besonders abgedrückt 1852, 4:49–60.

    Google Scholar 

  7. 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 

  8. Jennette,JC, Falk,RJ: Small Vessel Vasculitis. N Engl J Med 1997, 337:1512–1523. A review of the clinical manifestations, diagnosis, and treatment of the major forms of small-vessel vasculitis, with special emphasis on microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss syndrome. The authors advocate using a synthesis serologic and clinical information to reach an appropriate diagnosis.

    Article  PubMed  CAS  Google Scholar 

  9. Godman,G, Churg,J: Wegener’s granulomatosis. Pathology and review of the literature. Arch Pathol Lab Med 1954, 58:533–553.

    CAS  Google Scholar 

  10. Zeek,PM, Smith,CC, Weeter,JC: Studies on periarteritis nodosa: the differentiation between the vascular lesions of periarteritis nodosa and of hypersensitivity. Am J Pathol 1948, 24:889–917.

    PubMed  CAS  Google Scholar 

  11. Zeek,PM: Periarteritis nodosa: a critical review. Am J Clin Pathol 1952, 22:777–790.

    PubMed  CAS  Google Scholar 

  12. Klinger,H: Grenzformen der Periarteriitis nodosa. Frankf Ztschr Pathol 1931, 42:455–480.

    Google Scholar 

  13. Wegener,F: Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems under der Nieren. Beitr Pathol Anat 1939, 102:36–68.

    Google Scholar 

  14. Churg,J, Strauss,L: Allergic granulomatosis, allergic antiitis, and periarteritis nodosa. Am J Pathol 1951, 27:277–294.

    PubMed  CAS  Google Scholar 

  15. Kawasaki,T: MLNS showing particularskin desquamation from the finger and toe in infants. Allergy 1967, 16:178–189.

    PubMed  CAS  Google Scholar 

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

    Google Scholar 

  17. Shulman,ST, De,Inocenio J, Hirsch,R: Kawasaki disease. Ped Clin North Am 1995, 42:1205–22.

    CAS  Google Scholar 

  18. Willan R: On Cutaneous Diseases. vol.1. Kimber & Conrad; 1808:452–471.

  19. Schöonlein JL: Allegemeine und specielle Pathologie und Therapie, edn 3. Herisau, Germany, Literatur-Comptoir; 1837:48.

  20. Henoch,E: Uber den zusammenhang von purpura und intestinal-stoerungen. Berl Klin Wochenschur 1868, 5:517–519.

    Google Scholar 

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

  22. Osler,W: On the visceral complications of erythema eudativum multiforme. Am J Med Sci 1895, 2:629–647.

    Article  Google Scholar 

  23. Osler,W: The visceral lesions of purpura and allied conditions. Br Med J 1914, 1:517–525.

    Article  PubMed  Google Scholar 

  24. Goodpasture,WE: The significance of certain pulmonary lesions in relation to the etiology of influenza. Am J Med Sci 1919, 158:863–870.

    Article  Google Scholar 

  25. Martinez,JS, Kohler,PF: Variant "Goodpasture’s syndrome": the need for immunologic criteria in rapidly progressive glomerulonephritis and hemorrhagic pneumonitis. Ann Intern Med 1971, 75:67–76.

    PubMed  CAS  Google Scholar 

  26. Niles,JL, Bottinger,EP, Saurina,GR, et al.:The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med 1996, 156:440–445. The authors studies 88 patients with evidence of lung hemorrhage and nephritis. The largest group of patients had ANCA and not anti-GMB antibodies. The authors advocate testing for antimyeloperoxidase and antiproteinase 3 antibodies in all patients with pulmonary-renal vasculitic syndrome.

    Article  PubMed  CAS  Google Scholar 

  27. Baart de la Faille-Kuyper EH, Kater,L, et al.:IgA-deposits in cutaneous blood vessel walls and mesangium in Henoch-Schönlein syndrome. Lancet 1973, 1:892–893.

    Article  Google Scholar 

  28. Meltzer,M, Franklin,EC, Elias,K, McCluskey,RT: Cryoglobulinemia —a clinical and laboratory study. II. Cryoglobulins with rheumatoid factor activity. Am J Med 1966, 40:837–856.

    Article  PubMed  CAS  Google Scholar 

  29. Ronco,P, Verroust,P, Mignon,F, et al.:Immunopathological studies of polyarteritis nodosa and Wegener’s granulomatosis: a report of 43 patients with 51 renal biopsies. Quart J Med 1983, 52:212–223.

    PubMed  CAS  Google Scholar 

  30. Weiss,MA, Crissman,JD: Renal biopsy findings in Wegener’s granulomatosis: segmental necrotizing glomerulonephritis with glomerular thrombosis. Hum Pathol 1984, 15:943–956.

    Article  PubMed  CAS  Google Scholar 

  31. Jennette,JC: Anti-neutrophil cytoplasmic autoantibodyassociated disease: a pathologist’s perspective. Am J Kidney Dis 1991, 18:164–170.

    PubMed  CAS  Google Scholar 

  32. Gross,WL, Schmitt,WH, Csernok,E: ANCA and associated diseases: immunodiagnostic and pathogenetic aspects. Clin Exp Immunol 1993, 91:1–12.

    Article  PubMed  CAS  Google Scholar 

  33. Kallenberg,CGM, Brouwer,E, Weening,JJ, Cohen,Tervaert JW:Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiologic potential. Kidney Int 1994, 46:1–15.

    Article  PubMed  CAS  Google Scholar 

  34. Hagen,EC, Daha,MR, Hermans,J, et al.:Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis: EC/BCR Project for ANCA Assay Standardization. Kidney Int 1998, 53:796–798. Antineutrophil cytoplasmic autoantibodies (ANCA) results by immunofluorescence assay (IFA) and enzyme immunoassay (EIA) were evaluated in 169 newly diagnosed and 189 historical patients with vasculitis and were compared with results in 184 disease controls and 740 healthy controls. Diagnostic categorization was better when an EIA was used to detect ANCA. A significant minority of patients with Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome were negative for ANCA. Thus, a negative ANCA result does not rule out these diagnoses.

    Article  Google Scholar 

  35. Lim,LCL, Taylor,III JG, Schmitz,JL, et al.:Diagnostic usefulness of antineutrophil cytoplasmic autoantibody serology: comparative evaluation of commercial indirect fluorescent antibody kits and enzyme immunoassay kits. Am J Clin Pathol 1999, 111:363–369. The authors evaluated the diagnostic sensitivity, specificity, and predictive value of commercial indirect immunofluorescence assay (IFA) kits for cytoplasmic pattern ANCA (C-ANCA) and perinuclear pattern ANCA (P-ANCA), and commercial enzyme immunoassay (EIA) kits for proteinase 3 specific ANCA (PR3-ANCA) and myeloperoxidase specific ANCA (MPO-ANCA). Sera from 146 patients with pauciimmune glomerulonephritis vasculitis and 396 patients with other renal diseases were evaluated. The authors recommend using EIA for making diagnostic decisions and to show the influence of clinical presentations on the predictive value of ANCA testing.

    PubMed  CAS  Google Scholar 

  36. Hunder,GG, Arend,WP, Bloch,DA, et al.:The American College of Rheumatology 1990 criteria for the classification of vasculitis. Arthritis Rheum 1990, 33:1065–1067.

    Article  PubMed  CAS  Google Scholar 

  37. Rao,JK, Allen,NB, Pincus,T: Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis. Ann Intern Med 1998, 129:345–352. The authors used the 1990 American College of Rheumatology classification system to categorize 198 consecutive patients who presented with possible vasculitis. The ACR system did not perform well. Positive predictive values were unacceptably low for the categories of Wegener’s granulomatosis, giant cell arteritis, polyarteritis nodosa, and hypersensitivity angiitis.

    PubMed  CAS  Google Scholar 

  38. Gorevic,PD, Kassab,HJ, Levo,Y, et al.:Mixed cryoglobulinemia: clinical aspects and long-term follow-up of 40 patients. Am J Med 1980, 69:287–308.

    Article  PubMed  CAS  Google Scholar 

  39. Fauci,AS, Haynes,BF, Katz,P, Wolff,SM: Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983, 98:76–85.

    PubMed  CAS  Google Scholar 

  40. Goldstein,AR, White,RHR, Akuse,R, Chantler,C: Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 1992, 339:280–282.

    Article  PubMed  CAS  Google Scholar 

  41. Hoffman,GS, Kerr,GS, Leavitt,RY, et al.:Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 1992, 116:488–498.

    PubMed  CAS  Google Scholar 

  42. Bacon,PA: Therapy of vasculitis. J Rheumatol 1994, 21:788–790.

    PubMed  CAS  Google Scholar 

  43. Lhote,F, Guillevin,L: Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Rheum Dis Clin North Am 1995, 21:911–947.

    PubMed  CAS  Google Scholar 

  44. Nachman,PH, Hogan,SL, Jennette,JC, Falk,RJ: Treatment response and relapse in ANCA-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996, 7:33–39. The authors study the rate of remission, relapse, and treatment resistance in 107 patients with ANCA-positive microscopic polyangiitis or renal limited vasculitis. Induction and retention of remission was better with cyclophosphamide than with corticosteroids alone. The data indicate that early diagnosis and therapy are required for optimum outcome.

    PubMed  CAS  Google Scholar 

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Jennette, J.C., Falk, R.J. Do vasculitis categorization systems really matter?. Curr Rheumatol Rep 2, 430–438 (2000). https://doi.org/10.1007/s11926-000-0044-4

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  • DOI: https://doi.org/10.1007/s11926-000-0044-4

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