Search

Paola Galozzi, Sara Bindoli, Andrea Doria, Paolo Sfriso Rheumatology Unit, Department of Medicine DIMED, University of Padova, Padova, ItalyCorrespondence: Paola Galozzi, Rheumatology Unit, Department of Medicine DIMED, University of Padova, via Giustiniani, 2, Padova, 35128, Italy, Tel +39 049 821 8654, Email paola.galozzi@unipd.itAbstract: 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: biologics, AOSD, IL-1 inhibitors, IL-6 inhibitors, small molecules, new treatment

Systemic juvenile idiopathic arthritis (SJIA) is a disease marked with arthritis and several features of systemic inflammation including fevers, rashes, hepatosplenomegaly, lymphadenopathy, and serositis. The presentation can be variable and arthritis can be a later feature. Macrophage activation syndrome can be a life-threatening complication of this illness and requires early recognition and prompt therapy. Advancements in understanding the biology of SJIA have led to the development of cytokine-targeted therapies, mainly interleukin-1 (IL-1) and IL-6 inhibitors that have significantly improved outcomes. In this review, we provide an update on the advances in the understanding of SJIA biology and also the therapeutic options., Competing Interests: Disclosure Fellowship salary for Sriharsha Grevich is funded by Pfizer Medical Academic Partnerships grant. Susan Shenoi has served on speakers’ bureau and advisory board of Novartis. The authors report no other conflicts of interest in this work.