Pathogenesis of PR3-ANCA associated vasculitis.

Abstract

Wegener's Granulomatosis (WG) is closely associated with antineutrophil cytoplasmic autoantibodies (ANCA), particularly those directed to proteinase 3 (PR3). ANCA directed to myeloperoxidase (MPO) are associated with microscopic polyangiitis (MPA) and the Churg Strauss syndrome. PR3-ANCA associated vasculitis differs from MPO-ANCA associated vasculitis particularly in the occurrence of granulomatous inflammation and more widespread disease in the former condition. Studies in experimental animals strongly suggest a pathogenic role for MPO-ANCA, but this is less clear for PR3-ANCA. Here, we review pathogenic pathways in PR3-ANCA associated vasculitis focussing on unique patterns of PR3 expression on the neutrophil membrane in WG, as surface expression of PR3 is a prerequisite for stimulation by PR3-ANCA; (PR3-specific) cellular immunity in WG; and the role of Staphylococcus aureus carriage in this disease. These factors, together, may explain, at least in part, differences in clinical expression between MPO-ANCA and PR3-ANCA associated vasculitis.

• Authors: C G M Kallenberg
• Journal: Journal of autoimmunity (J Autoimmun) 2008 Feb-Mar Vol. 30 Issue 1-2 Pg. 29-36 ISSN: 0896-8411 [Print] England
• PMID: 18162369 (Publication Type: Journal Article, Review)

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Peroxidase
• Myeloblastin

Topics

• Animals
• Antibodies, Antineutrophil Cytoplasmic (immunology, metabolism)
• Churg-Strauss Syndrome (immunology)
• Granulomatosis with Polyangiitis (immunology)
• Humans
• Immunity, Cellular
• Myeloblastin (immunology, metabolism)
• Neutrophil Activation
• Neutrophils (enzymology, immunology, metabolism)
• Peroxidase (immunology, metabolism)
• Staphylococcus aureus (immunology)
• Vasculitis (immunology, physiopathology)