G.M. Hänsch and K. Andrassy

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Autoantibodies to neutrophil cytoplasmic antigens (ANCA) are associated with numerous chronic inflammatory diseases, particularly, but not exclusively, with primary vasculitides such as Wegener's granulomatosis, microscopic polyangiitis, or Churg Strauss syndrome. To discuss clinic, diagnosis, and therapeutic options as well as the pathogenesis of ANCA-associated primary vasculitides, an international conference was established the so-called "Vasculitis and ANCA-Workshop". In the book, important contributions from the last Workshop, held in June 2005 in Heidelberg, Germany, are presented.
A role of ANCA in the development and perpetuation of the disease is presumed – but still controversially discussed [Hänsch et al.]. Consequently research is focused mainly on the nature and possible function of the respective autoantigens, particularly of proteinase 3, the Wegener's autoantigen [Jenne and Kuhl; Vietinghoff et al.] and the possible role of the autoantibodies in vitro [Little et al.]. As an alternative to the mere function of autoantibodies, the participation of T-lymphocytes in the development of vasculitis is a long-term subject of research. In the paper by Kälsch et al., the seminal findings as well as the more recent data are summarised, and a concept including T-lymphocytes in other immunocompetent cells in the development of ANCA-associated vasculitis is discussed.
Finally, clinical aspects of ANCA-associated aspects are presented, including therapy regimens [de Groot and Jayne; Kötter et al.], as well as insights on the consequences of long-term immunosuppressive therapy [Lode and Schmidt-Ioanas].