Therapeutic Strategies in Newly Diagnosed Still’s Disease: Real-Life Clinicians’ Choices from the METAPHOR Project Worldwide Survey

Francesco Baldo¹, Greta Rogani², Claudia Bracaglia³, Dirk Foell⁴, Marco Gattorno⁵, Jordi anton⁶, Paul Brogan⁷, Scott Canna⁸, randy Cron⁹, Alexiei GROM¹⁰, Merav Heshin Bekenstein¹¹, Raju Khubchandani¹², Seza Özen¹³, Pierre Quartier¹⁴, Angelo Ravelli¹⁵, Grant Schulert¹⁶, Mao Mizuta¹⁷, Joost Swart¹⁸, Rashmi Sinha¹⁹, AnnaCarin Horne²⁰, Fabrizio De Benedetti²¹, Christiaan Scott²², Marija Jelusic²³, Masaki Shimizu²⁴, Bruno Fautrel²⁵, Nicolino Ruperto²⁶, Sebastiaan Vastert² and Francesca Minoia²⁷, ¹ASST-Pini-CTO, Milano, Milan, Italy, ²University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, ³IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Rome, Italy, ⁴Universisty Hospital of Muenster, Muenster, ⁵IRCCS G. Gaslini, Genova, Genoa, Italy, ⁶Hospital Sant Joan de Düu. Universitat de Barcelona, Barcelona, Spain, ⁷UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁸Children's Hospital of Philadelphia, Philadelphia, PA, ⁹University of Alabama at Birmingham, Birmingham, AL, ¹⁰Cincinnati Children’s Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ¹¹Tel Aviv Medical Center Israel, Binyamina, Tel Aviv, Israel, ¹²Jaslok Hospital and Research Center, Mumbai, India, ¹³Hacettepe University Medical Faculty, Ankara, Turkey, ¹⁴Hôpital Necker-Enfants Malades, Paris, France, ¹⁵IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Genoa, Italy, ¹⁶Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹⁷Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan, Kobe, Japan, ¹⁸Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Utrecht, Netherlands, ¹⁹Systemic JIA Foundation, Cincinnati, OH, ²⁰Karolinska University Hospital, Sollentuna, Sweden, ²¹Bambino Gesu Children's Hospital, Rome, Rome, Italy, ²²Childrens Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada, ²³University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ²⁴Tokyo Medical and Dental University, Bunkyo-ku, Kanazawa, Japan, ²⁵Sorbonne Université - APHP, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Inserm UMRS ¹¹³⁶-⁵, PARIS, France, Paris, France, ²⁶Université Milano Bicocca and Fondazione IRCSS S. Gerardo dei Tintori, Monza, Monza and Brianza, Italy, ²⁷Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy

Meeting: ACR Convergence 2025

Keywords: Access to care, Juvenile idiopathic arthritis, macrophage activation syndrome, Still's disease, Surveys

Session Information

Date: Tuesday, October 28, 2025

Title: (2124–2158) Pediatric Rheumatology – Clinical Poster III

Session Type: Poster Session C

Session Time: 10:30AM-12:30PM

Background/Purpose: Despite continuous advances in care and the recent publication of updated international recommendations, relevant discrepancies in the management of Still’s disease (SD) may still exist, due to different clinical presentation and practice setting. The aim of the study is to assess current intial treatment strategies in SD worldwide, and to identify factors influencing clinical decision-making.

Methods: As part of the METAPHOR project, a PReS/PRINTO initiative to optimize treatment in SD and macrophage activation syndrome, a global survey on SD treatment was developed and distributed (Dec 3, 2024–Feb 14, 2025). Topics were selected by 22 experts. The survey included demographic data, clinical practice insights, a patient-led section on unmet needs. International physicians part of the PReS/PRINTO network and adult rheumatologists involved in SD care were invited to complete the anonymous online survey (Dec 3, 2024–Feb 14, 2025).

Results: A total of 206 clinicians, mainly pediatric rheumatologists (91%), from 56 countries completed the survey. In newly diagnosed SD patients without MAS, 34% of respondents would initiate anti-IL1/IL6 biologic without glucocorticoids (GCs); the rest would use GCs alone (24%) or in combination with biologics (42%). Factors favoring GCs use included severe pericarditis (64%), severe arthritis (55%), and refractory disease risk factors (16%), as hyperferritinemia, lung involvement, early onset and Trisomy 21.Anakinra was the most frequently used biologic in 1st line (59%), followed by Tocilizumab (25%). Only 1.5% reported unavailability of any anti-IL1/IL-6 therapy. Factors driving anakinra’s choice were safety (72%), cost (41%), and predominant systemic phenotype (73%), while Tocilizumab was chosen for compliance (56%) and arthritis-dominant profile (80%). Difficult access to medication influenced decisions for Rilonacept (67%), Canakinumab (27%), Anakinra (17%); Tocilizumab was rarely unavailable (1.5%).In systemic-predominant SD, clinicians used NSAIDs (50%), GCs (46%), GCs pulses (42%),and Anakinra (45%) as first-line options. Second-line strategies included Tocilizumab (39%), GCs (31%), Anakinra (29%), GCs pulses (22%), and MTX (21%), while Tocilizumab (37%), JAK inhibitors (29%), and cyclosporin (24%) were the most selected options as third-line therapies. In SD patients with a prominent articular involvement at onset, half of the respondents would not change their therapeutic approach. Physician who modified thor strategy would use as a first line f therapies GCs (47%), oral steroids (44%), Tocilizumab (39%), MTX (34%), and intra-articular steroids (31%). Second-line choices were mostly Tocilizumab and MTX (~40%). Differences in management by clinical phenotype are detailed in Figure 1.

Conclusion: Still’s disease still represents a therapeutic challenge, mainly due to its heterogeneity in clinical expression. Our data reveals significant differences in treatment approaches, driven by clinical phenotype and drug availability. Future research is essential to optimize clustering of patients to foster tailored target treatments, while ensuring equitable access to effective therapies worldwide.

Disclosures: F. Baldo: None; G. Rogani: None; C. Bracaglia: None; D. Foell: None; M. Gattorno: Fresenius Kabi SwissBioSim GmbH, 6, Novartis, 1, 2, 5, 6, SOBI, 2, 6; J. anton: Sobi, 1, 5, 6; P. Brogan: Sobi, 6; S. Canna: AB2Bio, 2, Bristol-Myers Squibb(BMS), 2, Novartis, 2, Simcha Therapeutics, 12, In-kind provision of a reagent, Sobi, 6; r. Cron: AbbVie/Abbott, 1, American Board of Pediatrics, 12, Board Question Writer, AS2 Biotherapeutics, 2, CareerPhysician, 1, Neurogene, 2, Pfizer, 1, Sobia, 1, Springer, 9, VIDA Ventures, 2; A. GROM: National Institutes of Health, 5, Novartis, 2, 5, sJIA Foundation, 5, Sobi, 2, 5, Up-To-Date, 9; M. Heshin Bekenstein: None; R. Khubchandani: None; S. Özen: Novartis, 6, Pfizer, 6, Sobi, 6; P. Quartier: AbbVie, 2, 6, Chugai-Roche, 2, 6, Eli Lilly, 2, 6, Novartis, 2, 6, Pfizer, 2, 6, Sanofi, 1, 2, Sobi, 2, 6; A. Ravelli: AbbVie, 2, 5, 6, Alexion, 2, 5, 6, Bristol-Myers Squibb(BMS), 2, 5, 6, Galapagos, 2, 5, 6, Johnson & Johnson, 2, 5, 6, Novartis, 2, 5, 6, Pfizer, 2, 5, 6, Roche, 2, 5, 6, SOBI, 2, 5, 6; G. Schulert: IpiNovoyx, 5, Novartis, 2, SOBI, 2; M. Mizuta: None; J. Swart: None; R. Sinha: None; A. Horne: None; F. De Benedetti: AbbVie, 2, 5, Apollo, 2, 5, Elixiron, 2, 5, Kiniksa, 2, 5, Novartis, 2, 5, Sanofi, 2, 5, Sobi, 2, 5; C. Scott: None; M. Jelusic: None; M. Shimizu: None; B. Fautrel: AbbVie, 2, 5, Amgen, 2, Biogen, 2, BMS, 2, Celltrion, 2, Chugai, 2, Eli Lilly & Co., 2, 5, Fresenius Kabi, 2, Galapagos, 2, Janssen, 2, Medac, 2, MSD, 2, 5, Nordic Pharma, 2, Novartis, 2, OW KIN, 2, Pfizer, 2, 5, Roche, 2, Sandoz, 2, Sanofi-Genzyme, 2, SOBI, 2, UCB, 2, Viatris, 2; N. Ruperto: Abbvie, 2, 6, AClaris, 2, 6, AlfaSigma, 2, 6, Amgen, 2, 6, AstraZeneca, 2, 6, Aurinia, 2, 6, Boehringer-Ingelheim, 2, 6, Bristol Myers and Squibb, 2, 6, Eli Lilly, 2, 6, Galapagos, 2, 6, Genentech, 2, 6, Guidepoint, 2, 6, Idorsia, 2, 6, Janssen, 2, 6, Novartis, 2, 6, Pfizer, 2, 6, Roche, 2, 6, Sanofi, 2, 6, Takeda, 2, 6; S. Vastert: Novartis, 2, 6, Sobi, 2, 5, 6; F. Minoia: None.

To cite this abstract in AMA style:

Baldo F, Rogani G, Bracaglia C, Foell D, Gattorno M, anton J, Brogan P, Canna S, Cron r, GROM A, Heshin Bekenstein M, Khubchandani R, Özen S, Quartier P, Ravelli A, Schulert G, Mizuta M, Swart J, Sinha R, Horne A, De Benedetti F, Scott C, Jelusic M, Shimizu M, Fautrel B, Ruperto N, Vastert S, Minoia F. Therapeutic Strategies in Newly Diagnosed Still’s Disease: Real-Life Clinicians’ Choices from the METAPHOR Project Worldwide Survey [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/therapeutic-strategies-in-newly-diagnosed-stills-disease-real-life-clinicians-choices-from-the-metaphor-project-worldwide-survey/. Accessed .

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