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 C. 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 journalArticleAcademicpeer-review

2 Citations (Scopus)
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Abstract

Objectives:
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. The objectives of this study were 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 three rounds. In the first two rounds, online lists 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 effects, and preferred the use of biologics over conventional treatment. The role of IL-1 and IL-6 blocking agents was considered important in the treatment of AOSD.

Conclusion:
 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 languageEnglish
Pages (from-to)1656-1663
Number of pages8
JournalRheumatology (United Kingdom)
Volume63
Issue number6
DOIs
Publication statusPublished - 1 Jun 2024

Bibliographical note

Publisher Copyright:
© 2023 The Author(s).

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