T-lymphocytes and disease mechanisms in Wegener's granulomatosis.

The mechanisms underlying Wegener's granulomatosis (WG) are not well understood. The role of T-cells in the pathogenesis of WG has only recently come into focus of research. This review presents recent developments regarding the role of T-cells in WG. The occurrence of anti-neutrophil-cytoplasmic antibodies (ANCA) directed against proteinase-3 (PR-3) is a hallmark of WG. ANCA seem to mediate vasculitic damage in WG. Apart from ANCA, T-cells are involved in disease mechanisms. T-cells might participate in ANCA formation. Furthermore, T-cells are observed in affected tissue and granulomatous lesions. T-cells are indispensable for granuloma formation in other diseases and this might apply to WG too. In line with this, several aberrations of T-cell populations and alterations of the T-cell response were recently discovered in patients suffering from WG. Therefore, the impact of T-cell polarization, genotypic alterations modifying T-cell function and specific T-cell subsets on disease pathogenesis is discussed. Moreover, the influence of Staphylococcus aureus on T-cells and self-tolerance in WG is further elucidated. Finally, therapeutic options and implications with regard to T-cell-mediated pathogenesis are highlighted. Copyright (C) 2009 S. Karger AG, Basel