Department of Pathology.
Department of Immunohematology and Blood Transfusion, Leiden University Medical Center, Leiden, the Netherlands.
Curr Opin Rheumatol. 2019 Jan;31(1):3-8. doi: 10.1097/BOR.0000000000000557.
To provide a comprehensive overview of the current insight into the role of complement activation in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). In addition, the therapeutic options targeting the complement system in AAV are discussed.
It has become increasingly clear that complement, and more specifically signalling through the C5a receptor, contributes to the immunopathology of AAV. This has led to the design of clinical trials with a C5a receptor blocker. The first results show a reduction in tissue damage and a favourable safety profile, as other parts of the complement defence system are left intact.
Although AAV was initially regarded as a pauci-immune disease, it is now well established that, in addition to autoantibodies, complement plays an essential role in the disease process. Animal models delivered the first insight, but the effective therapeutic interventions using complement inhibitors provided the proof that indeed complement activation contributes to disease activity and tissue damage in human AAV.
全面概述补体激活在抗中性粒细胞胞质抗体相关性血管炎（AAV）中的作用。此外，还讨论了针对 AAV 补体系统的治疗选择。
越来越清楚的是，补体，更具体地说是通过 C5a 受体的信号转导，导致 AAV 的免疫病理学发生。这导致了针对 C5a 受体阻滞剂的临床试验的设计。最初的结果显示组织损伤减少和有利的安全性特征，因为补体防御系统的其他部分保持完整。
尽管 AAV 最初被认为是一种少免疫疾病，但现在已经确定，除了自身抗体外，补体在疾病过程中也起着至关重要的作用。动物模型提供了第一个见解，但使用补体抑制剂进行有效的治疗干预提供了证据，即补体激活确实导致了人类 AAV 的疾病活动和组织损伤。
