Imperial College Kidney and Transplant Institute, Imperial College, London, UK.
Ther Clin Risk Manag. 2010 Jun 24;6:253-64. doi: 10.2147/tcrm.s6112.
The antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are a spectrum of heterogeneous autoimmune diseases characterized by necrotizing small vessel vasculitis and the presence of ANCA. These chronic multisystem disorders may be life-threatening if there is major organ involvement, such as acute renal failure or pulmonary hemorrhage, and require significant initial immunosuppression and long-term maintenance treatment. Long-established protocols using cyclophosphamide and prednisolone have resulted in dramatically improved outcomes for patients since the 1970s. Subsequently, international collaboration has contributed to a growing evidence base and consensus in the management of these rare disorders. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, has maintained outcomes whilst decreasing toxicity. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. This review will explore the current evidence base for management of ANCA-associated vasculitis and future treatment prospects.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎是一组异质性自身免疫性疾病，其特征为坏死性小血管炎和 ANCA 阳性。如果存在主要器官受累，如急性肾衰竭或肺出血，这些慢性多系统疾病可能危及生命，需要初始大剂量免疫抑制和长期维持治疗。自 20 世纪 70 年代以来，使用环磷酰胺和泼尼松龙的既定方案显著改善了患者的预后。随后，国际合作在这些罕见疾病的管理方面积累了越来越多的证据和共识。通过使用硫唑嘌呤或甲氨蝶呤而非环磷酰胺来修改传统的治疗方案，以及引入利妥昔单抗等新型药物，在降低毒性的同时保持了疗效。然而，疗效不完全、感染和累积毒性等治疗局限性仍然存在。这些问题一直推动着使用靶向免疫疗法寻找更安全、更有效的免疫系统调节方法。本文将探讨目前管理 ANCA 相关性血管炎的循证医学证据和未来的治疗前景。
