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Review
. 2022 Apr 21:16:21-34.
doi: 10.2147/BTT.S290329. eCollection 2022.

Progress in Biological Therapies for Adult-Onset Still's Disease

Paola Galozzi  1 , Sara Bindoli  1 , Andrea Doria  1 , Paolo Sfriso  1
Affiliations
Review

Progress in Biological Therapies for Adult-Onset Still's Disease

Paola Galozzi et al. Biologics. .

Abstract

Adult-onset Still's disease (AOSD) is a rare multifactorial autoinflammatory disorder of unknown etiology, characterized by an excessive release of cytokines triggered by dysregulated inflammation and articular and systemic manifestations. The clinical spectrum of AOSD ranges from self-limiting forms with mild symptoms to life-threatening cases and presents clinical and biological similarities with the juvenile form (sJIA). Nowadays, the advances in biologic agents no longer limit the treatment to NSAIDs, glucocorticoids, or conventional synthetic DMARDs. The blockade of IL-1 and IL-6 is effective in the treatment of systemic and articular inflammation of AOSD patients; however, novel compounds with different properties and targets are now available and others are being studied. In this review, starting from the pathogenesis of AOSD, we summarized the current and emerging biological therapies, possible effective agents for achieving AOSD control and remission.

Keywords: AOSD; IL-1 inhibitors; IL-6 inhibitors; biologics; new treatment; small molecules.

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Conflict of interest statement

The authors report no conflicts of interest in this work.

Figures

Figure 1
Figure 1
Scheme of pathogenesis of AOSD and identification of targets for old (red), new (green), and proposed (light blue) biological therapy.
See this image and copyright information in PMC

References

    1. Sfriso P, Bindoli S, Galozzi P. Adult-onset still’s disease: molecular pathophysiology and therapeutic advances. Drugs. 2018;78(12):1187–1195. doi:10.1007/s40265-018-0956-9 - DOI - PubMed
    1. Mollaeian A, Chen J, Chan NN, et al. Adult onset Still’s disease in the elderly: a case-based literature review. BMC Rheumatol. 2021;5(1):12. doi:10.1186/s41927-021-00183-6 - DOI - PMC - PubMed
    1. Efthimiou P, Kontzias A, Hur P, et al. Adult-onset Still’s disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum. 2021;51(7):858–874. doi:10.1016/j.semarthrit.2021.06.004 - DOI - PubMed
    1. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19(3):424–430. - PubMed
    1. Fautrel B, Le Moël G, Saint-Marcoux B, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease. J Rheumatol. 2001;28(2):322–329. - PubMed