Pathways to ANCA production: From differentiation of dendritic cells by proteinase 3 to B lymphocyte maturation in Wegener's granuloma

Elena Csernok*, Frank Moosig, Wolgang L. Gross

*Corresponding author for this work
9 Citations (Scopus)

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.

Original languageEnglish
JournalClinical Reviews in Allergy and Immunology
Volume34
Issue number3
Pages (from-to)300-306
Number of pages7
ISSN1080-0549
DOIs
Publication statusPublished - 06.2008

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)

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