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Epitope-specific anti-neutrophil cytoplasmic antibodies: Do they matter? Can they be detected?


Ulrich Specks, Thoracic Diseases Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Stabile Bldg. 8-56, 200 First Street SW, Rochester, MN 55905, USA. e-mail:
The author declares no conflicts of interest.


Proteinase 3 (PR3)-anti-neutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase (MPO)-ANCA are suggested to play a pathogenic role as they are closely related to the small-vessel vasculitis syndromes, Wegener's granulomatosis and microscopic polyangiitis. A large body of in vitro and animal experiments supports this concept. The mechanisms of action involve a direct interaction between ANCA and its antigen. The epitope specificity of ANCA may therefore influence the functional effects of ANCA and/or may reflect the mechanisms behind different disease manifestations or disease courses.