Granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis belong to the anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitides (AAV). They share the feature of vasculitic manifestations in small to medium-size vessel beds which can occur in nearly any organ system. The treatment of AAV is dependent on stage and activity and is carried out on the basis of randomized controlled trials with an initial remission induction regimen followed by maintenance treatment. Apart from glucocorticoids, conventional immunosuppressants are the basis of treatment whereby biologics, such as rituximab have emerged as new treatment options.