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. 1998 Sep;79(3):246–250. doi: 10.1136/adc.79.3.246

Antineutrophil cytoplasmic antibodies in Wegener's granulomatosis

S Wong 1, V Shah 1, M Dillon 1
PMCID: PMC1717682  PMID: 9875021

Abstract

The prevalence of antineutrophil cytoplasmic antibodies (ANCA) was studied in 12 children with Wegener's granulomatosis. The serum samples were taken in the active phase of disease and were screened for ANCA by indirect immunofluorescence with normal neutrophils and enzyme linked immunosorbent assay (ELISA) using crude neutrophil extract, proteinase 3, myeloperoxidase, cathepsin G, lactoferrin, and elastase as antigens. Of these 12 patients, 10 were positive for ANCA in the active phase of their illness, and they showed a predominantly cytoplasmic ANCA staining pattern on indirect immunofluorescence. There were high titres of ANCA directed against crude neutrophil extract, proteinase 3, myeloperoxidase, and cathepsin G. IgM isotypes occurred as commonly as IgG isotypes. Therefore, screening for ANCA is usually but not invariably positive in children with Wegener's granulomatosis. Specific diagnosis still relies on clinical and pathological features, and the value of ANCA in the diagnosis of paediatric Wegener's granulomatosis requires further study.



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Selected References

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  1. Bajema I. M., Hagen E. C., van der Woude F. J., Bruijn J. A. Wegener's granulomatosis: a meta-analysis of 349 literary case reports. J Lab Clin Med. 1997 Jan;129(1):17–22. doi: 10.1016/s0022-2143(97)90157-8. [DOI] [PubMed] [Google Scholar]
  2. Egner W., Chapel H. M. Titration of antibodies against neutrophil cytoplasmic antigens is useful in monitoring disease activity in systemic vasculitides. Clin Exp Immunol. 1990 Nov;82(2):244–249. doi: 10.1111/j.1365-2249.1990.tb05434.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Ellis E. N., Wood E. G., Berry P. Spectrum of disease associated with anti-neutrophil cytoplasmic autoantibodies in pediatric patients. J Pediatr. 1995 Jan;126(1):40–43. doi: 10.1016/s0022-3476(95)70497-3. [DOI] [PubMed] [Google Scholar]
  4. Esnault V. L., Soleimani B., Keogan M. T., Brownlee A. A., Jayne D. R., Lockwood C. M. Association of IgM with IgG ANCA in patients presenting with pulmonary hemorrhage. Kidney Int. 1992 May;41(5):1304–1310. doi: 10.1038/ki.1992.194. [DOI] [PubMed] [Google Scholar]
  5. Gross W. L., Csernok E., Helmchen U. Antineutrophil cytoplasmic autoantibodies, autoantigens, and systemic vasculitis. APMIS. 1995 Feb;103(2):81–97. doi: 10.1111/j.1699-0463.1995.tb01083.x. [DOI] [PubMed] [Google Scholar]
  6. Jennette J. C., Falk R. J., Andrassy K., Bacon P. A., Churg J., Gross W. L., Hagen E. C., Hoffman G. S., Hunder G. G., Kallenberg C. G. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb;37(2):187–192. doi: 10.1002/art.1780370206. [DOI] [PubMed] [Google Scholar]
  7. Kerr G. S., Fleisher T. A., Hallahan C. W., Leavitt R. Y., Fauci A. S., Hoffman G. S. Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegener's granulomatosis. Arthritis Rheum. 1993 Mar;36(3):365–371. doi: 10.1002/art.1780360312. [DOI] [PubMed] [Google Scholar]
  8. Leavitt R. Y., Fauci A. S., Bloch D. A., Michel B. A., Hunder G. G., Arend W. P., Calabrese L. H., Fries J. F., Lie J. T., Lightfoot R. W., Jr The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990 Aug;33(8):1101–1107. doi: 10.1002/art.1780330807. [DOI] [PubMed] [Google Scholar]
  9. Lesavre P. Antineutrophil cytoplasmic autoantibodies antigen specificity. Am J Kidney Dis. 1991 Aug;18(2):159–163. doi: 10.1016/s0272-6386(12)80873-0. [DOI] [PubMed] [Google Scholar]
  10. Nash M. C., Dillon M. J. Antineutrophil cytoplasm antibodies and vasculitis. Arch Dis Child. 1997 Sep;77(3):261–264. doi: 10.1136/adc.77.3.261. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Nash M. C., Jones C. L., Walker R. G., Powell H. R. Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in children. Pediatr Nephrol. 1993 Feb;7(1):11–14. doi: 10.1007/BF00861551. [DOI] [PubMed] [Google Scholar]
  12. Pall A. A., Savage C. O. Mechanisms of endothelial cell injury in vasculitis. Springer Semin Immunopathol. 1994;16(1):23–37. doi: 10.1007/BF00196711. [DOI] [PubMed] [Google Scholar]
  13. Rao J. K., Weinberger M., Oddone E. Z., Allen N. B., Landsman P., Feussner J. R. The role of antineutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener granulomatosis. A literature review and meta-analysis. Ann Intern Med. 1995 Dec 15;123(12):925–932. doi: 10.7326/0003-4819-123-12-199512150-00005. [DOI] [PubMed] [Google Scholar]
  14. Rottem M., Fauci A. S., Hallahan C. W., Kerr G. S., Lebovics R., Leavitt R. Y., Hoffman G. S. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr. 1993 Jan;122(1):26–31. doi: 10.1016/s0022-3476(05)83482-1. [DOI] [PubMed] [Google Scholar]
  15. Savage C. O., Harper L., Adu D. Primary systemic vasculitis. Lancet. 1997 Feb 22;349(9051):553–558. doi: 10.1016/s0140-6736(97)80118-3. [DOI] [PubMed] [Google Scholar]
  16. Savage C. O., Winearls C. G., Jones S., Marshall P. D., Lockwood C. M. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis. Lancet. 1987 Jun 20;1(8547):1389–1393. doi: 10.1016/s0140-6736(87)90591-5. [DOI] [PubMed] [Google Scholar]
  17. Tervaert J. W., Huitema M. G., Hené R. J., Sluiter W. J., The T. H., van der Hem G. K., Kallenberg C. G. Prevention of relapses in Wegener's granulomatosis by treatment based on antineutrophil cytoplasmic antibody titre. Lancet. 1990 Sep 22;336(8717):709–711. doi: 10.1016/0140-6736(90)92205-v. [DOI] [PubMed] [Google Scholar]
  18. Thomas D. M., Moore R., Donovan K., Wheeler D. C., Esnault V. L., Lockwood C. M. Pulmonary-renal syndrome in association with anti-GBM and IgM ANCA. Lancet. 1992 May 23;339(8804):1304–1304. doi: 10.1016/0140-6736(92)91640-t. [DOI] [PubMed] [Google Scholar]
  19. Venning M. C., Quinn A., Broomhead V., Bird A. G. Antibodies directed against neutrophils (C-ANCA and P-ANCA) are of distinct diagnostic value in systemic vasculitis. Q J Med. 1990 Dec;77(284):1287–1296. doi: 10.1093/qjmed/77.3.1287. [DOI] [PubMed] [Google Scholar]
  20. Wiik A. S. Anti-neutrophil cytoplasmic antibodies in primary small vessel vasculitides. Scand J Rheumatol. 1996;25(2):65–69. doi: 10.3109/03009749609069209. [DOI] [PubMed] [Google Scholar]
  21. Wiik A. Delineation of a standard procedure for indirect immunofluorescence detection of ANCA. APMIS Suppl. 1989;6:12–13. [PubMed] [Google Scholar]
  22. van der Woude F. J., Rasmussen N., Lobatto S., Wiik A., Permin H., van Es L. A., van der Giessen M., van der Hem G. K., The T. H. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet. 1985 Feb 23;1(8426):425–429. doi: 10.1016/s0140-6736(85)91147-x. [DOI] [PubMed] [Google Scholar]

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