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. 2024 May 3;63(6):1656-1663.
doi: 10.1093/rheumatology/kead461.

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

Helen L Leavis  1 , Paul L A van Daele  2 , Catharina Mulders-Manders  3 , Renée Michels  4 , Abraham Rutgers  5 , Elizabeth Legger  6 , Marc Bijl  7 , Elisabeth A Hak  8 , Wai-Kwan Lam-Tse  9 , Femke Bonte-Mineur  10 , Peter Fretter  11 , Anna Simon  3 , Pieter van Paassen  12 , Marlies C van der Goes  13 , Marcel Flendrie  14 , Ward Vercoutere  15 , Antoine W T van Lieshout  16 , Arjen Leek  17 , Sebastiaan J Vastert  17 , Sander W Tas  8
Affiliations

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

Helen L Leavis et al. Rheumatology (Oxford). .

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.

Keywords: Adult-onset Still’s disease; Delphi; IL-1; Still’s disease; diagnosis; guideline; treatment.

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Figures

Figure 1.
Figure 1.
Diagnostic workup of (suspected) AOSD. AOSD: adult onset Still’s disease; GC: glucocorticoids; HLH: haemophagocytic lymphohistiocytosis; MAS: macrophage activation syndrome
Figure 2.
Figure 2.
Suggested treatment algorithm for patients with AOSD. Severe disease is defined as hospital admission needed, with organ involvement or life-threatening disease. Systemic symptoms: fever, weight loss, sore throat, skin rash, lymphadenopathy, hepatosplenomegaly. In case of predominant arthritis, IL-6 blockade may be preferred over IL-1 blockade. Low-dose glucocorticoid is ≤15 mg prednisone or equivalent. Double arrows indicate equivalent options and a decision should be made upon the physician’s discretion. GC refers to high dose glucocorticoid, i.e. prednisone 0.5–1.0 mg/kg or equivalent. ‘IL-1’ indicates IL-1 blockade; ‘IL-6’ indicates IL-6 blockade. MTX dosing according to EULAR; IL-1 blockade 100–300 mg subcutaneously or off label intravenously. AOSD: Adult onset Still’s Disease; GC: glucocorticoid; TNFi: TNF inhibitors
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References

    1. Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still's disease. J Autoimmun 2018;93:24–36. - PubMed
    1. Yoo DH. Treatment of adult-onset still's disease: up to date. Expert Rev Clin Immunol 2017;13:849–66. - PubMed
    1. Colafrancesco S, Manara M, Bortoluzzi A et al.; 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 2019;21:275. - PMC - PubMed
    1. Hasson F, Keeney S, McKenna H. Research guidelines for the Delphi survey technique. J Adv Nurs 2000;32:1008–15. - PubMed
    1. Humphrey-Murto S, Varpio L, Wood TJ et al. The use of the Delphi and other consensus group methods in medical education research: a review. Acad Med 2017;92:1491–8. - PubMed