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.
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.
Authors: James M Kelley; Paul A Monach; Chuanyi Ji; Yebin Zhou; Jianming Wu; Sumiaki Tanaka; Alfred D Mahr; Sharleen Johnson; Carol McAlear; David Cuthbertson; Simon Carette; John C Davis; Paul F Dellaripa; Gary S Hoffman; Nader Khalidi; Carol A Langford; Phillip Seo; E William St Clair; Ulrich Specks; John H Stone; Robert F Spiera; Steven R Ytterberg; Peter A Merkel; Jeffrey C Edberg; Robert P Kimberly Journal: Proc Natl Acad Sci U S A Date: 2011-12-06 Impact factor: 11.205
Authors: Gunnar Tomasson; Peter C Grayson; Alfred D Mahr; Michael Lavalley; Peter A Merkel Journal: Rheumatology (Oxford) Date: 2011-10-29 Impact factor: 7.580
Authors: John H Stone; Peter A Merkel; Robert Spiera; Philip Seo; Carol A Langford; Gary S Hoffman; Cees G M Kallenberg; E William St Clair; Anthony Turkiewicz; Nadia K Tchao; Lisa Webber; Linna Ding; Lourdes P Sejismundo; Kathleen Mieras; David Weitzenkamp; David Ikle; Vicki Seyfert-Margolis; Mark Mueller; Paul Brunetta; Nancy B Allen; Fernando C Fervenza; Duvuru Geetha; Karina A Keogh; Eugene Y Kissin; Paul A Monach; Tobias Peikert; Coen Stegeman; Steven R Ytterberg; Ulrich Specks Journal: N Engl J Med Date: 2010-07-15 Impact factor: 91.245
Authors: Sebastian Unizony; Miguel Villarreal; Eli M Miloslavsky; Na Lu; Peter A Merkel; Robert Spiera; Philip Seo; Carol A Langford; Gary S Hoffman; Cg M Kallenberg; E William St Clair; David Ikle; Nadia K Tchao; Linna Ding; Paul Brunetta; Hyon K Choi; Paul A Monach; Fernando Fervenza; John H Stone; Ulrich Specks Journal: Ann Rheum Dis Date: 2015-11-30 Impact factor: 19.103