Recently, the European League Against Rheumatism (EULAR) published recommendations on the management of patients with primary systemic vasculitides. The use of the given definitions for different activities and stages of the diseases is encouraged not only in clinical trails but also for the purpose of harmonisation in daily routine. Concerning the diagnostic work-up an interdisciplinary approach in co-operation with expert-centre is recommended. Therapy consists of remission induction and maintenance. For induction therapy in small vessel vasculitis cyclophosphamide or medium potent immunosuppressants such as methotrexate plus glucocorticoids should be used according to disease stage and activity. Glucocorticoids also represent the mainstay of remission induction in large vessel vasculitis. Additional immunosuppressants should be considered, if the disease can not be controlled by glucocorticoids or for the purpose of sparing glucocorticoids. Refractory disease should be treated within clinical trails. Further recommendations deal with monitoring of diseases activity and therapy as well as with measures to avoid complications and late sequelae.