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Review

What you should know about PR3-ANCA: An introduction

Allan Wiik

Author affiliations

Department of Autoimmunology, Statens Serum Institut, Copenhagen, Denmark

Citation and License

Arthritis Res 2000, 2:252-254  doi:10.1186/ar96

Published: 12 June 2000

Abstract

Anti-neutrophil cytoplasmic antibodies (ANCA) have become important diagnostic markers of small vessel vasculitides characterized by focal necrotizing lesions of vessel walls and accumulation of lymphocytes and macrophages around the affected vessels. IgG class ANCA directed to proteinase 3 (PR3) of neutrophils and monocytes seem to be directly involved in the pathophysiology of vascular damage by causing excessive neutrophil activation and vessel wall destruction. PR3 and elastase are important players in the mechanisms of vascular necrosis. Methods of detecting ANCA have now been defined but are not uniformly used, even though clinical decisions heavily depend on correct ANCA results.

Keywords:
anti-neutrophil cytoplasm antibodies; immunopathology; proteinase 3; systemic vasculitis