Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts

Helen L Leavis; Paul L A van Daele; Catharina Mulders-Manders; Renée Michels; Abraham Rutgers; Elizabeth Legger; Marc Bijl; Elisabeth A Hak; Wai-Kwan Lam-Tse; Femke Bonte-Mineur; Peter Fretter; Anna Simon; Pieter van Paassen; Marlies van der Goes; Marcel Flendrie; Ward Vercoutere; Antoine W T van Lieshout; Arjen Leek; Sebastiaan J Vastert; Sander W Tas

doi:10.1093/rheumatology/kead461

Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts

Helen L Leavis^*, Paul L A van Daele, Catharina Mulders-Manders, Renée Michels, Abraham Rutgers, Elizabeth Legger, Marc Bijl, Elisabeth A Hak, Wai-Kwan Lam-Tse, Femke Bonte-Mineur, Peter Fretter, Anna Simon, Pieter van Paassen, Marlies van der Goes, Marcel Flendrie, Ward Vercoutere, Antoine W T van Lieshout, Arjen Leek, Sebastiaan J Vastert, Sander W Tas

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

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Abstract

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis. Most doctors treating AOSD in the Netherlands treat <5 patients per year. Currently, there is no internationally accepted treatment guideline for AOSD.

OBJECTIVES: To conduct a Delphi panel aimed at reaching consensus about diagnostic and treatment strategies for patients with AOSD and to use the outcomes as a basis for a treatment algorithm.

METHODS: The Delphi panel brought together 18 AOSD experts: rheumatologists, internists and paediatricians. The Delphi process consisted of 3 rounds. In the first two rounds, online list of questions and statements were completed. In the third round, final statements were discussed during a virtual meeting and a final vote took place. Consensus threshold was set at 80%. Two targeted literature searches were performed identifying the level of evidence of the consensus-based statements.

RESULTS: Consensus was reached on 29 statements, including statements related to diagnosis and diagnostic tests, definition of response and remission, the therapy, the use of methotrexate, and tapering of treatment. The panel consented on reduction of the use of glucocorticoids to avoid side-effect, and preferred the use of biologics over conventional treatment. The role of interleukin-1 and interleukin-6 blocking agents was considered important in the treatment of AOSD.

CONCLUSIONS: In this Delphi panel, a high level of consensus was achieved on recommendations for diagnosis and therapy of AOSD that can serve as a basis for a treatment guideline.

Original language	English
Pages (from-to)	1656–1663
Number of pages	8
Journal	Rheumatology
Volume	63
Issue number	6
Early online date	5-Sept-2023
DOIs	https://doi.org/10.1093/rheumatology/kead461
Publication status	Published - Jun-2024

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Leavis, H. L., van Daele, P. L. A., Mulders-Manders, C., Michels, R., Rutgers, A., Legger, E., Bijl, M., Hak, E. A., Lam-Tse, W.-K., Bonte-Mineur, F., Fretter, P., Simon, A., van Paassen, P., van der Goes, M., Flendrie, M., Vercoutere, W., van Lieshout, A. W. T., Leek, A., Vastert, S. J., & Tas, S. W. (2024). Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts. Rheumatology, 63(6), 1656–1663. https://doi.org/10.1093/rheumatology/kead461

@article{874a047d14db4577987899be163b66af,

title = "Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts",

abstract = "BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis. Most doctors treating AOSD in the Netherlands treat <5 patients per year. Currently, there is no internationally accepted treatment guideline for AOSD.OBJECTIVES: To conduct a Delphi panel aimed at reaching consensus about diagnostic and treatment strategies for patients with AOSD and to use the outcomes as a basis for a treatment algorithm.METHODS: The Delphi panel brought together 18 AOSD experts: rheumatologists, internists and paediatricians. The Delphi process consisted of 3 rounds. In the first two rounds, online list of questions and statements were completed. In the third round, final statements were discussed during a virtual meeting and a final vote took place. Consensus threshold was set at 80%. Two targeted literature searches were performed identifying the level of evidence of the consensus-based statements.RESULTS: Consensus was reached on 29 statements, including statements related to diagnosis and diagnostic tests, definition of response and remission, the therapy, the use of methotrexate, and tapering of treatment. The panel consented on reduction of the use of glucocorticoids to avoid side-effect, and preferred the use of biologics over conventional treatment. The role of interleukin-1 and interleukin-6 blocking agents was considered important in the treatment of AOSD.CONCLUSIONS: In this Delphi panel, a high level of consensus was achieved on recommendations for diagnosis and therapy of AOSD that can serve as a basis for a treatment guideline.",

author = "Leavis, {Helen L} and {van Daele}, {Paul L A} and Catharina Mulders-Manders and Ren{\'e}e Michels and Abraham Rutgers and Elizabeth Legger and Marc Bijl and Hak, {Elisabeth A} and Wai-Kwan Lam-Tse and Femke Bonte-Mineur and Peter Fretter and Anna Simon and {van Paassen}, Pieter and {van der Goes}, Marlies and Marcel Flendrie and Ward Vercoutere and {van Lieshout}, {Antoine W T} and Arjen Leek and Vastert, {Sebastiaan J} and Tas, {Sander W}",

note = "{\textcopyright} The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.",

year = "2024",

month = jun,

doi = "10.1093/rheumatology/kead461",

language = "English",

volume = "63",

pages = "1656–1663",

journal = "Rheumatology",

issn = "1462-0324",

publisher = "Oxford University Press",

number = "6",

}

Leavis, HL, van Daele, PLA, Mulders-Manders, C, Michels, R, Rutgers, A , Legger, E, Bijl, M, Hak, EA, Lam-Tse, W-K, Bonte-Mineur, F, Fretter, P, Simon, A, van Paassen, P, van der Goes, M, Flendrie, M, Vercoutere, W, van Lieshout, AWT, Leek, A, Vastert, SJ & Tas, SW 2024, 'Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts', Rheumatology, vol. 63, no. 6, pp. 1656–1663. https://doi.org/10.1093/rheumatology/kead461

TY - JOUR

T1 - Management of adult-onset Still's disease

T2 - evidence- and consensus-based recommendations by experts

AU - Leavis, Helen L

AU - van Daele, Paul L A

AU - Mulders-Manders, Catharina

AU - Michels, Renée

AU - Rutgers, Abraham

AU - Legger, Elizabeth

AU - Bijl, Marc

AU - Hak, Elisabeth A

AU - Lam-Tse, Wai-Kwan

AU - Bonte-Mineur, Femke

AU - Fretter, Peter

AU - Simon, Anna

AU - van Paassen, Pieter

AU - van der Goes, Marlies

AU - Flendrie, Marcel

AU - Vercoutere, Ward

AU - van Lieshout, Antoine W T

AU - Leek, Arjen

AU - Vastert, Sebastiaan J

AU - Tas, Sander W

PY - 2024/6

Y1 - 2024/6

N2 - BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis. Most doctors treating AOSD in the Netherlands treat <5 patients per year. Currently, there is no internationally accepted treatment guideline for AOSD.OBJECTIVES: To conduct a Delphi panel aimed at reaching consensus about diagnostic and treatment strategies for patients with AOSD and to use the outcomes as a basis for a treatment algorithm.METHODS: The Delphi panel brought together 18 AOSD experts: rheumatologists, internists and paediatricians. The Delphi process consisted of 3 rounds. In the first two rounds, online list of questions and statements were completed. In the third round, final statements were discussed during a virtual meeting and a final vote took place. Consensus threshold was set at 80%. Two targeted literature searches were performed identifying the level of evidence of the consensus-based statements.RESULTS: Consensus was reached on 29 statements, including statements related to diagnosis and diagnostic tests, definition of response and remission, the therapy, the use of methotrexate, and tapering of treatment. The panel consented on reduction of the use of glucocorticoids to avoid side-effect, and preferred the use of biologics over conventional treatment. The role of interleukin-1 and interleukin-6 blocking agents was considered important in the treatment of AOSD.CONCLUSIONS: In this Delphi panel, a high level of consensus was achieved on recommendations for diagnosis and therapy of AOSD that can serve as a basis for a treatment guideline.

AB - BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis. Most doctors treating AOSD in the Netherlands treat <5 patients per year. Currently, there is no internationally accepted treatment guideline for AOSD.OBJECTIVES: To conduct a Delphi panel aimed at reaching consensus about diagnostic and treatment strategies for patients with AOSD and to use the outcomes as a basis for a treatment algorithm.METHODS: The Delphi panel brought together 18 AOSD experts: rheumatologists, internists and paediatricians. The Delphi process consisted of 3 rounds. In the first two rounds, online list of questions and statements were completed. In the third round, final statements were discussed during a virtual meeting and a final vote took place. Consensus threshold was set at 80%. Two targeted literature searches were performed identifying the level of evidence of the consensus-based statements.RESULTS: Consensus was reached on 29 statements, including statements related to diagnosis and diagnostic tests, definition of response and remission, the therapy, the use of methotrexate, and tapering of treatment. The panel consented on reduction of the use of glucocorticoids to avoid side-effect, and preferred the use of biologics over conventional treatment. The role of interleukin-1 and interleukin-6 blocking agents was considered important in the treatment of AOSD.CONCLUSIONS: In this Delphi panel, a high level of consensus was achieved on recommendations for diagnosis and therapy of AOSD that can serve as a basis for a treatment guideline.

U2 - 10.1093/rheumatology/kead461

DO - 10.1093/rheumatology/kead461

M3 - Article

C2 - 37669122

SN - 1462-0324

VL - 63

SP - 1656

EP - 1663

JO - Rheumatology

JF - Rheumatology

IS - 6

ER -

Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts

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