Anti-neutrophil cytoplasmic antibodies-associated vasculitides (AAVs) are a heterogenous group of inflammatory diseases which primarily involve small vessels and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). They present heterogeneous clinical manifestations, while their diagnosis and management still remain a challenge for clinicians.

Nowadays, the treatment is based on two different regimens: the remission-induction treatment and the remission-maintenance treatment.

The therapeutic has grown over the years, with the aim to lessen adverse effects, improve quality of life of patients and maintain the disease under control. Biological treatments are the future: they act on different pathogenic pathways and may offer in the future a personalized management approach tailored to actual clinical manifestations.

The latest guidelines were published in 2015 by the European League Against Rheumatism (EULAR) and still represent the for the management of AAVs.

In this review, we will focus on the principal strategies to treat AAVs. We discuss the remission-induction therapy and the remission- maintenance therapy; we have also distinguished the management of GPA and MPA from that of EGPA, because of their different clinical pictures.