EULAR and the ERA–EDTA have published updated recommendations for the management of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Their recommendations include use of immunosuppressive therapy combined with glucocorticoids for remission induction; cyclosphosphamide and rituximab are considered to have similar efficacy. Plasma exchange is recommended for patients with rapidly progressive renal failure or severe diffuse pulmonary haemorrhage.