ANCA-associated Vasculitis Advances in Pathogenesis and Treatment

A role of ANCA in the development and perpetuation of vasculitis 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].