ANCA Are Detectable in Nearly All Patients with Active Severe Wegener's Granulomatosis

Javier D. Finkielman, Augustine S. Lee, Amber M. Hummel, Margaret A. Viss, Gregory L. Jacob, Henry A. Homburger, Tobias D Peikert, Gary S. Hoffman, Peter A. Merkel, Robert Spiera, E. William St. Clair, John C. Davis, W. Joseph McCune, Andrea K. Tibbs, Steven R Ytterberg, John H. Stone, Ulrich Specks

Research output: Contribution to journalArticle

165 Citations (Scopus)

Abstract

Background: The pathogenic significance of antineutrophilic cytoplasmic antibodies (ANCA) in Wegener's granulomatosis is controversial. Their presence is influenced by the extent, severity, and activity of the disease at the time of sampling. The objective of this study was to determine the frequency of ANCA in patients with active Wegener's granulomatosis and to assess the influence of disease severity on test results. Methods: Baseline serum samples from the 180 participants in a multicentric prospective trial were tested for ANCA by indirect immunofluorescence, direct enzyme-linked immunosorbent assay (ELISA), and capture ELISA. Disease activity was measured using the Birmingham Vasculitis Activity Score for Wegener's granulomatosis. All patients had active disease at enrollment. Patients were categorized as having severe (n = 128) or limited (n = 52) Wegener's granulomatosis. Results: When all ANCA detection methods were combined, 166 patients (92%) were ANCA positive, including 96% with severe disease and 83% with limited disease. Conclusion: ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis, but approximately 1 of 5 patients with active limited disease are ANCA negative. Immunofluorescence and both direct and capture ELISAs are required for optimal detection, suggesting that ANCA are not recognized equally well by all testing methods.

Original languageEnglish (US)
JournalAmerican Journal of Medicine
Volume120
Issue number7
DOIs
StatePublished - Jul 2007

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Granulomatosis with Polyangiitis
Antibodies
Enzyme-Linked Immunosorbent Assay
Direct Fluorescent Antibody Technique
Indirect Fluorescent Antibody Technique
Vasculitis
Serum

Keywords

  • Antineutrophil cytoplasmic antibodies
  • Enzyme-linked immunosorbent assay
  • Fluorescent antibody technique
  • Wegener's granulomatosis

ASJC Scopus subject areas

  • Nursing(all)

Cite this

ANCA Are Detectable in Nearly All Patients with Active Severe Wegener's Granulomatosis. / Finkielman, Javier D.; Lee, Augustine S.; Hummel, Amber M.; Viss, Margaret A.; Jacob, Gregory L.; Homburger, Henry A.; Peikert, Tobias D; Hoffman, Gary S.; Merkel, Peter A.; Spiera, Robert; St. Clair, E. William; Davis, John C.; McCune, W. Joseph; Tibbs, Andrea K.; Ytterberg, Steven R; Stone, John H.; Specks, Ulrich.

In: American Journal of Medicine, Vol. 120, No. 7, 07.2007.

Research output: Contribution to journalArticle

Finkielman, JD, Lee, AS, Hummel, AM, Viss, MA, Jacob, GL, Homburger, HA, Peikert, TD, Hoffman, GS, Merkel, PA, Spiera, R, St. Clair, EW, Davis, JC, McCune, WJ, Tibbs, AK, Ytterberg, SR, Stone, JH & Specks, U 2007, 'ANCA Are Detectable in Nearly All Patients with Active Severe Wegener's Granulomatosis', American Journal of Medicine, vol. 120, no. 7. https://doi.org/10.1016/j.amjmed.2006.08.016
Finkielman, Javier D. ; Lee, Augustine S. ; Hummel, Amber M. ; Viss, Margaret A. ; Jacob, Gregory L. ; Homburger, Henry A. ; Peikert, Tobias D ; Hoffman, Gary S. ; Merkel, Peter A. ; Spiera, Robert ; St. Clair, E. William ; Davis, John C. ; McCune, W. Joseph ; Tibbs, Andrea K. ; Ytterberg, Steven R ; Stone, John H. ; Specks, Ulrich. / ANCA Are Detectable in Nearly All Patients with Active Severe Wegener's Granulomatosis. In: American Journal of Medicine. 2007 ; Vol. 120, No. 7.
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abstract = "Background: The pathogenic significance of antineutrophilic cytoplasmic antibodies (ANCA) in Wegener's granulomatosis is controversial. Their presence is influenced by the extent, severity, and activity of the disease at the time of sampling. The objective of this study was to determine the frequency of ANCA in patients with active Wegener's granulomatosis and to assess the influence of disease severity on test results. Methods: Baseline serum samples from the 180 participants in a multicentric prospective trial were tested for ANCA by indirect immunofluorescence, direct enzyme-linked immunosorbent assay (ELISA), and capture ELISA. Disease activity was measured using the Birmingham Vasculitis Activity Score for Wegener's granulomatosis. All patients had active disease at enrollment. Patients were categorized as having severe (n = 128) or limited (n = 52) Wegener's granulomatosis. Results: When all ANCA detection methods were combined, 166 patients (92{\%}) were ANCA positive, including 96{\%} with severe disease and 83{\%} with limited disease. Conclusion: ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis, but approximately 1 of 5 patients with active limited disease are ANCA negative. Immunofluorescence and both direct and capture ELISAs are required for optimal detection, suggesting that ANCA are not recognized equally well by all testing methods.",
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AU - Finkielman, Javier D.

AU - Lee, Augustine S.

AU - Hummel, Amber M.

AU - Viss, Margaret A.

AU - Jacob, Gregory L.

AU - Homburger, Henry A.

AU - Peikert, Tobias D

AU - Hoffman, Gary S.

AU - Merkel, Peter A.

AU - Spiera, Robert

AU - St. Clair, E. William

AU - Davis, John C.

AU - McCune, W. Joseph

AU - Tibbs, Andrea K.

AU - Ytterberg, Steven R

AU - Stone, John H.

AU - Specks, Ulrich

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AB - Background: The pathogenic significance of antineutrophilic cytoplasmic antibodies (ANCA) in Wegener's granulomatosis is controversial. Their presence is influenced by the extent, severity, and activity of the disease at the time of sampling. The objective of this study was to determine the frequency of ANCA in patients with active Wegener's granulomatosis and to assess the influence of disease severity on test results. Methods: Baseline serum samples from the 180 participants in a multicentric prospective trial were tested for ANCA by indirect immunofluorescence, direct enzyme-linked immunosorbent assay (ELISA), and capture ELISA. Disease activity was measured using the Birmingham Vasculitis Activity Score for Wegener's granulomatosis. All patients had active disease at enrollment. Patients were categorized as having severe (n = 128) or limited (n = 52) Wegener's granulomatosis. Results: When all ANCA detection methods were combined, 166 patients (92%) were ANCA positive, including 96% with severe disease and 83% with limited disease. Conclusion: ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis, but approximately 1 of 5 patients with active limited disease are ANCA negative. Immunofluorescence and both direct and capture ELISAs are required for optimal detection, suggesting that ANCA are not recognized equally well by all testing methods.

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