Being an ADVOCATE for People with ANCA Vasculitis.

作者：H. Anders, Daigo Nakazawa · 发表于：American Society of Nephrology. Clinical Journal · 年份：2021 · DOI：10.2215/cjn.03670321 · 被引用次数：5 · 研究领域：Medicine

ANCA-associated vasculitis is a systemic autoimmune disease affecting numerous organs and tissues (1). Traditionally, the anti-inflammatory effects of glucocorticosteroids rapidly induce remission, and drugs like cyclophosphamide or rituximab suppress adaptive immunity to control andprevent relapse of the underlying autoimmune disease activity (1). Infections, diabetes, fractures, and other classic steroid toxicity keep contributing significantly to themorbidity andmortality of patientswithANCAvasculitis (2). Replacing steroids in the treatment of ANCA vasculitis would require drugs with equipotent and immediate anti-inflammatory effects, while sparing interferences with host defense andmetabolic homeostasis. The solutionwas to identify molecular targets within the innate immune system because innate immunity largely contributes to autoimmune tissue injury (3). The complement system has long been considered in this context because it is not involved in metabolic control, but it is a key mediator in many forms of sterile inflammation, including autoimmune vasculitis (4). However, the complement system is also critically involved in host defense. It took decades of research to identify the optimal molecular target within the complex cascades of complement activation and regulation (4,5). In 2017, the phase 2 Study to Evaluate the Safety and Efficacy of CCX168 in Subjects with ANCA-Associated Vasculitis (CLEAR) included patients with ANCA vasculitis receiving a standard inductio...