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Patients with adult-onset Still's disease have markedly elevated serum levels of proinflammatory cytokines, such as IL-1β, IL-6, and IL-18, suggesting the potential of these molecules as therapeutic targets. IL-6 accelerates macrophage and cytotoxic T-cell differentiation and neutrophil and macrophage chemotaxis and is one of the most important cytokines in the pathogenesis of adult-onset Still's disease. The review summarizes the importance of IL-6 in the pathogenesis of adult-onset Still's disease and clinical aspects of IL-6 inhibition from retrospective and prospective studies. Adult-onset Still's disease is a systemic inflammatory disease of unknown etiology and characterized by elevated various proinflammatory cytokines. In particular, serum concentrations of IL-6 is significantly high in patients with active adult-onset Still's disease, and many case reports, cohort studies and one randomized, placebo-controlled trail have shown the efficacy of IL-6 blockade in patients with adult-onset Still's disease who were refractory to glucocorticoids and other immunosuppressive treatments. IL-6 inhibition is effective for both systemic and joint manifestations with arthritis improving slowly. There is still a concern over the triggering of macrophage activation syndrome; however, the IL-6 inhibition strategy has introduced better management of adult-onset Still's disease.


Yuko Kaneko, Tsutomu Takeuchi. Interleukin-6 inhibition: a therapeutic strategy for the management of adult-onset Still's disease. Expert opinion on biological therapy. 2022 Jan;22(1):79-85

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PMID: 34126828

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