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What is new in systemic vasculitis?
  1. Department of Clinical Immunology
  2. University Hospital Groningen
  3. The Netherlands
  4. Department of Clinical Immunology
  5. University Hospital Maastricht
  6. The Netherlands
  1. Professor C G M Kallenberg, Department of Clinical Immunology, University Hospital Groningen, Hanzeplein 1, 9713 GZ Groningen, The Netherlands C.G.M.Kallenberg{at}

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A report from the 9th International Vasculitis/ANCA Workshop

From 12 to 15 April 2000 the 9th International Vasculitis/ANCA Workshop was held at the University Hospital Groningen, The Netherlands.1 This series of workshops started as an informal ANCA directed workshop in 1989 in Copenhagen and has grown into an international meeting dealing with the whole spectrum of primary systemic vasculitides, including ANCA associated vasculitis. The present workshop was attended by more than 300 delegates from 19 countries.

Endothelium-leucocyte interactions

The first part of the workshop dealt with endothelium-leucocyte interaction and the role of autoantibodies in this process. Mayet (Mainz, Germany) reviewed the studies of his group showing that endothelial cells, but also other cells like tubular epithelial cells and lung epithelium, express proteinase 3 (PR3), the main target antigen of antineutrophil cytoplasmic antibodies (ANCA) in Wegener's granulomatosis. Expression was shown not only at the protein level but also at the mRNA level. Stimulation with proinflammatory cytokines resulted in a strong expression of PR3 at the surface of endothelial cells, making PR3 accessible for circulating PR3-ANCA. Indeed, PR3-ANCA were able to upregulate the expression of adhesion molecules and the production of interleukin 8 (IL8) by endothelial cells that were prestimulated with proinflammatory cytokines such as tumour necrosis factor α. Daha et al (Leiden, The Netherlands) were not able to confirm PR3 expression by endothelial cells. In contrast, they demonstrated a PR3-binding molecule of 111 kDa on the membrane of endothelial cells. Adding PR3, both in its enzymatically active and inactive form, to endothelial cells resulted in enhanced production of IL8, a strong chemoattractant for neutrophils, and of MCP-1, which is chemotactive for monocytes and T cells. In addition, incubation of endothelial cells with PR3 resulted in upregulation of endothelial leucocyte adhesion molecules. Also, dose dependent apoptosis and cytolysis of endothelial cells was seen …

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