The treatment of adult-onset Still's disease with anakinra, a recombinant human IL-1 receptor antagonist: a systematic review of literature.

Roberto Giacomelli, Jurgen Sota, Piero Ruscitti, Corrado Campochiaro, Serena Colafrancesco, Lorenzo Dagna, Daniela Iacono, Florenzo Iannone, Giuseppe Lopalco, Paolo Sfriso, Luca Cantarini

Clinical and experimental rheumatology 2021 Jan-Feb

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Adult-onset Still's disease (AOSD) is a rare, inflammatory disease of unknown aetiology, generally affecting young adults and requiring immunosuppressive treatment. In the last few years, bio- logic disease-modifying anti-rheumatic drugs (bDMARDs) have been successfully used in refractory cases, based on the pathogenic role of inflammatory cytokines in AOSD. Amongst bDMARDs, several observations confirmed the clinical usefulness of anakinra, a recombinant human non-glycosylated IL-1 receptor antagonist, in AOSD. At present, the treatment is still largely empirical and due to the possible fallacious aspects of clinical judgement, in this work, we performed a systematic review of literature (SRL) to summarise the evidence regarding the treatment with anakinra in AOSD, analysing rate of complete remission, corticosteroids (CCSs)-sparing effect, long-term retention rate, and safety. After screening titles, abstracts and analysis of full text, 15 manuscripts were analysed: 1 open randomised multicentre trial with two parallel groups and 14 observational single-arm retrospective studies. Collectively, results of the present SRL suggest the effectiveness of anakinra in the treatment of patients with AOSD. Furthermore, patients with AOSD are likely to achieve a good clinical response with anakinra and these outcomes are associated with a largely favourable safety profile. Furthermore, the majority of patients treated with anakinra may achieve a complete remission, also in monotherapy. Finally, the treatment with anakinra is associated with an important CCSs-sparing effect, and, a large percentage of these patients may stop CCSs, thus reducing predictable long-term CCSs side effects without the occurrence of new flares.

Citation

Roberto Giacomelli, Jurgen Sota, Piero Ruscitti, Corrado Campochiaro, Serena Colafrancesco, Lorenzo Dagna, Daniela Iacono, Florenzo Iannone, Giuseppe Lopalco, Paolo Sfriso, Luca Cantarini. The treatment of adult-onset Still's disease with anakinra, a recombinant human IL-1 receptor antagonist: a systematic review of literature. Clinical and experimental rheumatology. 2021 Jan-Feb;39(1):187-195