Abstract: As a common systemic autoimmune disease, anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) may cause an impairment of kidney, lung and other important organs. Clinical cure is rather difficult. The previous therapeutic strategies have failed to provide long-term efficacy for patients so that there are lower standards of living. The incidence and prevalence of AAV has spiked in recent years. So how to prevent and manage AAV is still a hot topic. The exact etiology of AAV remains elusive and its recurrence is influenced by many factors. Growing studies have demonstrated that polygenic predisposition, epigenetic effects and environmental risk factors play some complex roles in the pathogenesis of AAV. As suggested by previous related foreign researches, patients with genetic susceptibility are more prone to AAV under the impact of environmental factors and environmental factors have some effects on the recurrence of AAV. This review summarized the latest researches of the impact of environment-related risk factors on the incidence of ANCA-associated vasculitis.