Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Mod Rheumatol. 2022 Jan 5;32(1):12-15. doi: 10.1093/mr/roab004.
Adult-onset Still's disease is a systemic inflammatory disease characterized by high spiking fever, arthritis, evanescent skin rash, leukocytosis, and hyperferritinemia. The pathogenesis of adult-onset Still's disease has not been fully understood yet; however, multiple proinflammatory cytokines, such as IL-1β and IL-6, play important roles in the development of adult-onset Still's disease. IL-6 is a multifunctional cytokine that accelerates the differentiation of macrophages and cytotoxic T-cells and chemotaxis of neutrophils and macrophages. Serum concentrations of IL-6 well correlate with disease activity of adult-onset Still's disease, and blockade of IL-6 has been proven to be effective in active adult-onset Still's disease. This review will focus on the recent understanding of the role of proinflammatory cytokines of adult-onset Still's disease and the efficacy of IL-6 inhibitors for the treatment of adult-onset Still's disease.
成人Still 病是一种全身炎症性疾病,其特征为高热、关节炎、一过性皮疹、白细胞增多和铁蛋白血症。成人Still 病的发病机制尚未完全阐明;然而,多种促炎细胞因子,如白细胞介素-1β和白细胞介素-6,在成人Still 病的发展中起重要作用。白细胞介素-6 是一种多功能细胞因子,可加速巨噬细胞和细胞毒性 T 细胞的分化以及中性粒细胞和巨噬细胞的趋化作用。血清白细胞介素-6 浓度与成人Still 病的疾病活动度密切相关,阻断白细胞介素-6 已被证明对活动性成人Still 病有效。本综述将重点介绍对成人Still 病促炎细胞因子作用的最新认识以及白细胞介素-6 抑制剂治疗成人Still 病的疗效。