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Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts

BACKGROUND: Adult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Because of its rarity, clinical trials are inherently small and often uncontrolled. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors i...

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Autores principales: Colafrancesco, Serena, Manara, Maria, Bortoluzzi, Alessandra, Serban, Teodora, Bianchi, Gerolamo, Cantarini, Luca, Ciccia, Francesco, Dagna, Lorenzo, Govoni, Marcello, Montecucco, Carlomaurizio, Priori, Roberta, Ravelli, Angelo, Sfriso, Paolo, Sinigaglia, Luigi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6907145/
https://www.ncbi.nlm.nih.gov/pubmed/31829244
http://dx.doi.org/10.1186/s13075-019-2021-9
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author Colafrancesco, Serena
Manara, Maria
Bortoluzzi, Alessandra
Serban, Teodora
Bianchi, Gerolamo
Cantarini, Luca
Ciccia, Francesco
Dagna, Lorenzo
Govoni, Marcello
Montecucco, Carlomaurizio
Priori, Roberta
Ravelli, Angelo
Sfriso, Paolo
Sinigaglia, Luigi
author_facet Colafrancesco, Serena
Manara, Maria
Bortoluzzi, Alessandra
Serban, Teodora
Bianchi, Gerolamo
Cantarini, Luca
Ciccia, Francesco
Dagna, Lorenzo
Govoni, Marcello
Montecucco, Carlomaurizio
Priori, Roberta
Ravelli, Angelo
Sfriso, Paolo
Sinigaglia, Luigi
author_sort Colafrancesco, Serena
collection PubMed
description BACKGROUND: Adult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Because of its rarity, clinical trials are inherently small and often uncontrolled. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors in the management of patients with AOSD, based on the best evidence and expert opinion. METHODS: A panel of 10 experts (9 rheumatologists and 1 pediatrician) was established. The first step was dedicated to a comprehensive literature review and development of statements. Two separate literature searches were performed on the MEDLINE (Pubmed), EMBASE, and BIOSIS databases through April 2018 to identify (1) differences and similarities between AOSD and pediatric Still’s disease (systemic juvenile idiopathic arthritis [SJIA]) and (2) the efficacy and safety of IL-1 inhibitors in AOSD treatment. In the second step, the statements were submitted in a Delphi process to a panel of 67 rheumatologists. Consensus threshold was set at 66%: positive, > 66% of voters selected scores 3 to 5; negative, > 66% of voters selected scores 1 or 2. In the third step, the voting results were analyzed, and the statements were finalized. RESULTS: Eleven statements were developed. Forty-six of 67 rheumatologists (72%) participated in the Delphi process. A positive consensus was reached after the first round of voting and was full (> 95%) on the majority of statements. A large consensus was achieved in considering AOSD and SJIA as the same disease. The use of anti-IL-1 therapies in refractory patients was considered quite safe and effective both as the first and as a subsequent line of biologic treatment, especially in systemic patients. Because of the lack of head-to-head comparisons, a different profile of efficacy among IL-1 inhibitors could not be established. There was a large consensus that failure of the first IL-1 inhibitor does not preclude response to another one. The lack of studies comparing early versus late treatment did not allow to draw conclusions; however, data from SJIA suggest a better response in early treatment. CONCLUSIONS: The Delphi method was used to develop recommendations that we hope will help clinicians in the management of patients with AOSD refractory to conventional therapies.
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spelling pubmed-69071452019-12-20 Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts Colafrancesco, Serena Manara, Maria Bortoluzzi, Alessandra Serban, Teodora Bianchi, Gerolamo Cantarini, Luca Ciccia, Francesco Dagna, Lorenzo Govoni, Marcello Montecucco, Carlomaurizio Priori, Roberta Ravelli, Angelo Sfriso, Paolo Sinigaglia, Luigi Arthritis Res Ther Research Article BACKGROUND: Adult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Because of its rarity, clinical trials are inherently small and often uncontrolled. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors in the management of patients with AOSD, based on the best evidence and expert opinion. METHODS: A panel of 10 experts (9 rheumatologists and 1 pediatrician) was established. The first step was dedicated to a comprehensive literature review and development of statements. Two separate literature searches were performed on the MEDLINE (Pubmed), EMBASE, and BIOSIS databases through April 2018 to identify (1) differences and similarities between AOSD and pediatric Still’s disease (systemic juvenile idiopathic arthritis [SJIA]) and (2) the efficacy and safety of IL-1 inhibitors in AOSD treatment. In the second step, the statements were submitted in a Delphi process to a panel of 67 rheumatologists. Consensus threshold was set at 66%: positive, > 66% of voters selected scores 3 to 5; negative, > 66% of voters selected scores 1 or 2. In the third step, the voting results were analyzed, and the statements were finalized. RESULTS: Eleven statements were developed. Forty-six of 67 rheumatologists (72%) participated in the Delphi process. A positive consensus was reached after the first round of voting and was full (> 95%) on the majority of statements. A large consensus was achieved in considering AOSD and SJIA as the same disease. The use of anti-IL-1 therapies in refractory patients was considered quite safe and effective both as the first and as a subsequent line of biologic treatment, especially in systemic patients. Because of the lack of head-to-head comparisons, a different profile of efficacy among IL-1 inhibitors could not be established. There was a large consensus that failure of the first IL-1 inhibitor does not preclude response to another one. The lack of studies comparing early versus late treatment did not allow to draw conclusions; however, data from SJIA suggest a better response in early treatment. CONCLUSIONS: The Delphi method was used to develop recommendations that we hope will help clinicians in the management of patients with AOSD refractory to conventional therapies. BioMed Central 2019-12-11 2019 /pmc/articles/PMC6907145/ /pubmed/31829244 http://dx.doi.org/10.1186/s13075-019-2021-9 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
Colafrancesco, Serena
Manara, Maria
Bortoluzzi, Alessandra
Serban, Teodora
Bianchi, Gerolamo
Cantarini, Luca
Ciccia, Francesco
Dagna, Lorenzo
Govoni, Marcello
Montecucco, Carlomaurizio
Priori, Roberta
Ravelli, Angelo
Sfriso, Paolo
Sinigaglia, Luigi
Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts
title Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts
title_full Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts
title_fullStr Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts
title_full_unstemmed Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts
title_short Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts
title_sort management of adult-onset still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of italian experts
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6907145/
https://www.ncbi.nlm.nih.gov/pubmed/31829244
http://dx.doi.org/10.1186/s13075-019-2021-9
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