Objective: In recent years,an increasing number of drugs have been proved to be associated with the induction of anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).This article reviews the latest research progress on drug-induced AAV.Data sources: We conducted a comprehensive and detailed search of the PubMed database.The search terms mainly included drug-induced,ANCA,and vasculitis.Study selection: We summarized the original articles and reviews on drug-induced AAV in recent years.The extracted information included the definition,epidemiology,associated drugs,pathogenesis,clinical features,diagnosis,treatment,and prognosis of drug-induced AAV.We also focused on the differences between drug-induced AAV and primary vasculitis.Results: The offending drugs leading to drug-induced AAV are almost from pharmacologic categories and we need to be vigilant when using these drugs.The pathogenesis of drug-induced AAV might be multifactorial.The formation of neutrophil extracellular traps is an important mechanism for the development of drug-induced AAV.The clinical features of drug-induced AAV are similar to those of primary AAV.Understanding the difference between drug-induced AAV and primary AAV is helpful to identify drug-induced AAV.Stopping the offending drug at once after diagnosis may be sufficient for those patients with mild symptoms.Immunosuppressive therapy should only be used in patients with vital organs involvement.Conclusions: Patients with drug-induced AAV usually have a good prognosis if they stop using the offending drug immediately.Recent advances in research on AAV are expected to help us better understand the pathogenesis of drug-induced AAV.