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Pathways to ANCA Production: From Differentiation of Dendritic Cells by Proteinase 3 to B Lymphocyte Maturation in Wegener’s Granuloma

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Abstract

Wegener’s granulomatosis, microscopic polyangiitis, and Churg–Strauss syndrome are idiopathic systemic vasculitides in which circulating anti-neutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 (PR3) or myeloperoxidase (MPO) are commonly found. Within the last 25 years, these antibodies were subject of intensive studies, and a growing body of evidence arose for a distinct role of ANCA in the pathogenesis of the ANCA-associated vasculitides (AAV). Yet, the evidence derived from clinical observations and in vitro studies remains circumstantial. The various animal models have provided substantial support for a pathogenic role of MPO–ANCA in vivo, but the debate if ANCA play a primary role in the pathogenesis of these diseases is still open. The aim of this review was to update current basic and clinical research on ANCA in the pathophysiology of AAV and to point out and discuss limitations and inconsistencies of the clinical and experimental evidence.

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Acknowledgment

This study was supported by BMBF grant no. 01 G1 9951, Competence network systemic inflammatory rheumatic diseases and Verein zur Förderung der Erforschung und Bekämpfung rheumatischer Erkrankungen Bad Bramstedt e.V.

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Authors and Affiliations

  1. Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Germany

    Elena Csernok, Frank Moosig & Wolgang L. Gross

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  1. Elena Csernok
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  2. Frank Moosig
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  3. Wolgang L. Gross
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Correspondence to Elena Csernok.

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Csernok, E., Moosig, F. & Gross, W.L. Pathways to ANCA Production: From Differentiation of Dendritic Cells by Proteinase 3 to B Lymphocyte Maturation in Wegener’s Granuloma. Clinic Rev Allerg Immunol 34, 300–306 (2008). https://doi.org/10.1007/s12016-007-8056-8

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  • DOI: https://doi.org/10.1007/s12016-007-8056-8

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