Reviews
The treatment of adult-onset Still’s disease with anakinra, a recombinant human IL-1 receptor antagonist: a systematic review of literature
R. Giacomelli1, J. Sota2, P. Ruscitti3, C. Campochiaro4, S. Colafrancesco5, L. Dagna6, D. Iacono7, F. Iannone8, G. Lopalco9, P. Sfriso10, L. Cantarini11
- Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, Italy. roberto.giacomelli@cc.univaq.it
- Research Center of Systemic Autoinflammatory Diseases, Behçet’s Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy.
- Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, Italy.
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University, Milan, Italy.
- Rheumatology Unit, Dipartimento di Scienze Cliniche Internistiche Anestesiologiche e Cardiovascolari Sapienza University, Rome, Italy.
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University, Milan, Italy.
- Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Campania “Luigi Vanvitelli”, Naples, Italy.
- Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Italy.
- Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Italy.
- Department of Medicine - DIMED, University of Padova, Italy.
- Research Center of Systemic Autoinflammatory Diseases, Behçet’s Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy.
CER13141
2021 Vol.39, N°1
PI 0187, PF 0195
Reviews
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PMID: 32452353 [PubMed]
Received: 26/01/2020
Accepted : 07/04/2020
In Press: 12/05/2020
Published: 05/02/2021
Abstract
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.
