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proLékaře.cz / Journals / Czech Rheumatology / 2021 - 4

. IL-1 inhibition in the treatment of systemic juvenile arthritis and adult-onset Still's disease

Czech version

Authors: H. Malcová 1;  T. Milota 1,2;  Z. Střížová 2;  D. Cebecauerová 1;  R. Horváth 1,2
Authors‘ workplace: Oddělení revmatologie dětí a dospělých 2. LF UK a FN Motol, Praha 1;  Ústav imunologie 2. LF UK a FN Motol, Praha 2
Published in: Čes. Revmatol., 29, 2021, No. 4, p. 209-227.
Category: Review Article

Overview

Polygenic autoinflammatory diseases (AIDs) typically include systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD). These diseases are characterized by the dysregulation of the innate immune responses with an excessive and uncontrolled overproduction of IL-1β in particular. Administration of the IL-1 blockers leads to a rapid improvement of the clinical symptoms, disease activity reduction, normalization of the laboratory parameters, and the possibility of reducing or even discontinuing glucocorticoid use. Most of the current data are focused on the therapeutic use of anakinra – an IL-1 receptor antagonist, canakinumab – an anti-IL-1β monoclonal antibody, and rilonacept – a soluble decoy receptor. However, other promising agents, such as gevokizumab – IL-1β blocking monoclonal antibody, tadekinig alfa – a human recombinant IL-18-binding protein, and tranilast – an analog of a tryptophan metabolite, are currently being tested. In this review article, the authors focus on the role of IL-1 and related cytokines in the regulation of the inflammatory responses in AIDs and provide a detailed review of clinical trials with IL-1 blockade in sJIA and AOSD, focusing on the efficacy and safety data.

Keywords:

canakinumab – anakinra – rilonacept – adult onset Still's disease (AOSD) – autoinflammatory diseases – interleukin-1 (IL-1) – systemic juvenile idiopathic arthritis (sJIA)

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