Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are disorders characterized by necrotizing inflammation of small sized vessels. The starring character is the neutrophil who triggers an immune response influenced by genetics and environmental factors. AAV is an uncommon disease, most information is based on European and North American population. There is spare information about epidemiologic characteristics of AAV in Latin America, however the multiethnicity, geographic diversity, low-income condition and use of toxic agents in agriculture and mining, may be factors that impact on clinical phenotype.