Open Access Open Badges Research article

Proteinase-3 as the major autoantigen of c-ANCA is strongly expressed in lung tissue of patients with Wegener's granulomatosis

Holger Brockmann1, Andreas Schwarting1, Jörg Kriegsmann2, Peter Petrow2, Andreas Gaumann2, Klaus-Michael Müller3, Peter Robert Galle1 and Werner Mayet4*

Author Affiliations

1 Department of Medicine, University of Mainz, Mainz, Germany

2 Institute of Pathology, University of Mainz, Mainz, Germany

3 Institute of Pathology, Professional Associations Hospital, Ruhr-University, Bochum, Germany

4 Center of Internal Medicine, Nordwest Hospital, Sanderbusch, Germany

For all author emails, please log on.

Arthritis Res 2002, 4:220-225  doi:10.1186/ar410

Published: 27 March 2002


Proteinase-3 (PR-3) is a neutral serine proteinase present in azurophil granules of human polymorphonuclear leukocytes and serves as the major target antigen of antineutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA) in Wegener's granulomatosis (WG). The WG disease appears as severe vasculitis in different organs (e.g. kidney, nose and lung). Little is known about the expression and distribution of PR-3 in the lung. We found that PR-3 is expressed in normal lung tissue and is upregulated in lung tissue of patients with WG. Interestingly, the parenchymal cells (pneumocytes type I and II) and macrophages, and not the neutrophils, express PR-3 most strongly and may contribute to lung damage in patients with WG via direct interaction with antineutrophil cytoplasmic antobodies (ANCA). These findings suggest that the PR-3 expression in parenchymal cells of lung tissue could be at least one missing link in the etiopathogenesis of pulmonary pathology in ANCA-associated disease.

granuloma; in situ hybridization; pneumocytes; proteinase-3; Wegener's granulomatosis