伴器官损害或威胁生命的ANCA相关性血管炎的治疗进展及挑战

随着大量临床研究的开展和各国指南的发布，伴有器官损害或威胁生命的ANCA相关性血管炎（AAV）的治疗已经获得了重要突破，从致死性疾病转变为一类慢性疾病。利妥昔单抗（RTX）在AAV诱导和维持治疗中的作用获得了有力的循证医学支持，并最终纳入现行的大部分指南。AAV患者的长期预后不仅取决于血管炎疾病本身，还取决于长期用药带来的不良反应。因此，优化治疗药物、治疗剂量和治疗周期，减少药物的不良反应，最终改善生存率是未来临床研究的工作方向。

Challenges and Progress in the Treatment of Organ Damage Associated by ANCA or Life-threatening ANCA Associated Vasculitis

With the development of a large number of clinical studies and the release of national guidelines，the treatment of organ damage associated by ANCA and ANCA associated vasculitis （AAV） has made an important breakthrough，transforming it from a fatal disease into a chronic disease. The role of rituximab （RTX）in induction and maintenance therapy has been strongly supported by evidence-based medicine and it has finally been included in most of the current guidelines. Optimizing treatment drugs，treatment doses and treatment cycles，reducing adverse drug reactions，and ultimately improving patient survival rates are the directions of future clinical research.