Author/Authors :
Javier D. Finkielman، نويسنده , , Augustine S. Lee، نويسنده , , Amber M. Hummel، نويسنده , , Margaret A. Viss، نويسنده , , Gregory L. Jacob، نويسنده , , Henry A. Homburger، نويسنده , , Tobias Peikert، نويسنده , , Gary S. Hoffman، نويسنده , , Peter A. Merkel، نويسنده , , Robert Spiera، نويسنده , , E. William St Clair، نويسنده , , John C. Davis Jr.، نويسنده , , W. Joseph McCune، نويسنده , , Andrea K. Tibbs، نويسنده , , Steven R. Ytterberg، نويسنده , , John H. Stone، نويسنده , , Ulrich Specks and WGET Research Group (see Appendix)، نويسنده ,
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.
