Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts
Abstract 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 inhi...
| Main Authors: | , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2019-12-01
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| Series: | Arthritis Research & Therapy |
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| Online Access: | https://doi.org/10.1186/s13075-019-2021-9 |
| _version_ | 1818724247529324544 |
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| author | Serena Colafrancesco Maria Manara Alessandra Bortoluzzi Teodora Serban Gerolamo Bianchi Luca Cantarini Francesco Ciccia Lorenzo Dagna Marcello Govoni Carlomaurizio Montecucco Roberta Priori Angelo Ravelli Paolo Sfriso Luigi Sinigaglia AOSD Consensus Group |
| author_facet | Serena Colafrancesco Maria Manara Alessandra Bortoluzzi Teodora Serban Gerolamo Bianchi Luca Cantarini Francesco Ciccia Lorenzo Dagna Marcello Govoni Carlomaurizio Montecucco Roberta Priori Angelo Ravelli Paolo Sfriso Luigi Sinigaglia AOSD Consensus Group |
| author_sort | Serena Colafrancesco |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-17T21:23:23Z |
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| institution | Directory Open Access Journal |
| issn | 1478-6362 |
| language | English |
| last_indexed | 2024-12-17T21:23:23Z |
| publishDate | 2019-12-01 |
| publisher | BMC |
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| series | Arthritis Research & Therapy |
| spelling | doaj.art-239df6f344fb4100a7cba331e7fc544f2022-12-21T21:32:06ZengBMCArthritis Research & Therapy1478-63622019-12-0121112110.1186/s13075-019-2021-9Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian expertsSerena Colafrancesco0Maria Manara1Alessandra Bortoluzzi2Teodora Serban3Gerolamo Bianchi4Luca Cantarini5Francesco Ciccia6Lorenzo Dagna7Marcello Govoni8Carlomaurizio Montecucco9Roberta Priori10Angelo Ravelli11Paolo Sfriso12Luigi Sinigaglia13AOSD Consensus GroupDipartimento di Scienze Cliniche, Internistiche, Anestesiologiche e Cardiovascolari, Rheumatology Unit, Sapienza University of RomeDivision of Rheumatology, ASST Gaetano Pini-CTORheumatology, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria di FerraraSC Reumatologia, ASL3 - Azienda Sanitaria GenoveseSC Reumatologia, ASL3 - Azienda Sanitaria GenoveseDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena, Policlinico “Le Scotte”Rheumatology, Dipartimento di Medicina di Precisione, Università della Campania “L. Vanvitelli”Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific InstituteRheumatology, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria di FerraraDepartment of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of PaviaDipartimento di Scienze Cliniche, Internistiche, Anestesiologiche e Cardiovascolari, Rheumatology Unit, Sapienza University of RomeClinica Pediatrica e Reumatologia, Istituto Giannina Gaslini and Università degli Studi di GenovaRheumatology Unit, Department of Medicine, University of PaduaDivision of Rheumatology, ASST Gaetano Pini-CTOAbstract 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.https://doi.org/10.1186/s13075-019-2021-9Adult-onset Still’s diseaseAnakinraCanakinumabInterleukin-1RilonaceptStill’s disease |
| spellingShingle | Serena Colafrancesco Maria Manara Alessandra Bortoluzzi Teodora Serban Gerolamo Bianchi Luca Cantarini Francesco Ciccia Lorenzo Dagna Marcello Govoni Carlomaurizio Montecucco Roberta Priori Angelo Ravelli Paolo Sfriso Luigi Sinigaglia AOSD Consensus Group Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts Arthritis Research & Therapy Adult-onset Still’s disease Anakinra Canakinumab Interleukin-1 Rilonacept Still’s disease |
| 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 | Adult-onset Still’s disease Anakinra Canakinumab Interleukin-1 Rilonacept Still’s disease |
| url | https://doi.org/10.1186/s13075-019-2021-9 |
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