中文摘要：

抗中性粒细胞胞浆抗体相关性血管炎（AAV）是一组以小血管壁的炎症及纤维素样坏死为特点的系统性自身免疫性疾病。其发病机制尚不明确，目前认为与遗传、环境、免疫等多因素共同作用有关。其中，遗传因素是AAV发病的一个重要因素。现就AAV遗传学方面研究进展予以综述。

英文摘要：

Antineutrophil cytoplasmic autoantibodies （ANCA）-associated vasculitis （AAV） is a group of systemic vasculitis strongly associated with ANCA. The disorders are characterized by necrotizing inflammation of small and medium size vessels. Genetic, environmental, and immunological factors contribute to the etiology and pathogenesis of AAV. Summarizing genetic basis in the pathogenesis of AAV is of significance.