抗嗜中性白血球相關的血管炎(Anti-neutrophil Cytoplasmic Autoantibody-Associated Vasculitides, AAV) 雖然罕見且原因尚未明瞭，但在過去的幾十年中相關的研究一直非常活躍。AAV 屬於小管徑血管炎，臨床症狀多樣並且影響多種器官，如腎和肺等。AAV 與其他自體免疫性疾病相似，在具有誘發性遺傳背景且暴露於致病環境因素的患者中發生。AAV 的致病機轉，包含其體內的病理組織與臨床表現，常常涉及抗嗜中性白血球抗體(Anti-neutrophil Cytoplasmic Autoantibody, ANCA) 誘發的中性白血球過度活化，並伴隨炎性細胞因子的分泌。臨床診斷與治療AAV 時，應避免延誤治療以預防疾病伴隨之器官損害，特別是要注意其腎功能的變化，因為可能導致慢性腎衰竭而需長期洗腎。而由於治療AAV 時，常有不良反應且容易復發，因此需要長期耐心的追蹤，病患方能獲得適當的病情控制。

Although antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) rarity and still unknown causes, research on those vasculitides has been very active over the past few decades. AAV are small-vessel vasculitides and the clinical symptoms vary and affect several organs, such as the kidney and lung. AAV is similar to other autoimmune diseases and develops in patients with a predisposing genetic background who have been exposed to causative environmental factors. AAV pathogenic mechanisms should explain the pathologic and clinical manifestations of AAV, which involves ANCA-mediated neutrophil overactivations that subsequently release inflammatory cytokines. AAV clinical diagnosis and treatment should avoid delay to prevent end organ damage, particularly renal disease. Adverse responses to treatment AAV are common, as are relapses, so requiring long term follow-up is needed.