ANCA相关性血管炎的诊疗进展

The Development of Diagnosis and Treatment for ANCA-associated Vasculitis

抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)是一种常见的自身免疫性原发性小血管炎,主要侵犯小血管,如毛细血管、小静脉、小动脉和微动脉,以血管坏死性炎症与破坏为病理特征,肾、肺、皮肤、五官、神经系统等全身多个器官或系统均可受累。AAV的发病率日益增高,中国的流行病学数据仍缺乏。其病因及发病机制至今未明,地域不同,临床表现不同且多样,目前尚无统一的诊断标准。早期诊断及合理治疗可提高患者生存率和生活质量。

ANCA-associated vasculitis( AAV) is a common autoimmune primary vasculitis that mainly invades small blood vessels,such as capillaries,venules,arterioles and arterioles,with vascular necrotic inflammation and destruction as pathological features. Multiple organ systems such as the kidney,lungs,skin,facial features,and nervous system can be affected. The incidence of AAV is increasing,but epidemiological datas in China are still facing the deficiency problem. The etiology and pathogenesis of this disease are still unknown. Different regions lead to different clinical manifestations. There is no unified diagnostic standard. Early diagnosis and reasonable treatment can improve survival rates and the quality of life.