ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2138

Factors driving therapeutic decision-making in Still’s Disease: When to Start and When to Stop? Data from the METAPHOR Project Worldwide Survey

Francesco Baldo1, Greta Rogani2, Claudia Bracaglia3, Dirk Foell4, Marco Gattorno5, Marija Jelusic6, Jordi Anton7, Paul Brogan8, Scott Canna9, Randy Cron10, Fabrizio De Benedetti11, Alexei Grom12, Merav Heshin Bekenstein13, AnnaCarin Horne14, Raju Khubchandani15, Mao Mizuta16, Seza Özen17, Pierre Quartier Dit Maire18, Angelo Ravelli19, Masaki Shimizu20, Grant Schulert12, Christiaan Scott21, Rashmi Sinha22, Nicolino Ruperto23, Joost Swart24, Bruno Fautrel25, Sebastiaan Vastert2 and Francesca Minoia26, 1ASST-Pini-CTO, Milano, Milan, Italy, 2University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, 3IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Rome, Italy, 4University Hospital Muenster, Muenster, Germany, 5IRCCS G. Gaslini, Genova, Genoa, Italy, 6University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, 7Hospital Sant Joan de Düu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, 8UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 9Children's Hospital of Philadelphia, Philadelphia, PA, 10University of Alabama at Birmingham, Birmingham, AL, 11Bambino Gesu Children's Hospital, Rome, Rome, Italy, 12Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 13Tel Aviv Medical Center Israel, Binyamina, Tel Aviv, Israel, 14Karolinska University Hospital, Sollentuna, Sweden, 15SRCC Childrens Hospital Mumbai, Mumbai, India, 16Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan, Kobe, Japan, 17Hacettepe University Medical Faculty, Ankara, Turkey, 18Necker hospital, Paris Cedex 15, France, 19IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Genoa, Italy, 20Tokyo Medical and Dental University, Bunkyo-ku, Kanazawa, Japan, 21Childrens Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada, 22Systemic JIA Foundation, Cincinnati, OH, 23Université Milano Bicocca and Fondazione IRCSS S. Gerardo dei Tintori, Monza, Monza and Brianza, Italy, 24Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Utrecht, Netherlands, 25Sorbonne Université - APHP, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Inserm UMRS 1136-5, PARIS, France, Paris, France, 26Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy

Meeting: ACR Convergence 2025

Keywords: , , , ,

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 improvement in care and the recent update of international recommendations, relevant discrepancies in the diagnostic and treatment approach to Still’s disease (SD) may still exist, in particular in the timing and factors influencing treatment start and withdraw. The study was aimed to explore current global treatment strategies for SD, focusing on parameters driving the decision-making process at treatment start and at withdrawal

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 based. Topics were selected by 22 experts, including 1 patient representative and 1 adult rheumatologist. 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 responses, mainly from pediatric rheumatologists (91%) from 56 countries were collected. Infectious tests performed at SD onset included TB, EBV, and blood cultures (80%), parvovirus, HBV, HCV, and SARS-CoV-2 (50%). Most physicians performed peripheral blood smears (85%), while 54% bone marrow aspiration, 38% immunophenotyping and 17% bone marrow biopsy. Imaging was commonly performed, with chest X-ray, abdominal ultrasound, and echocardiography as the most frequent investigations (~80%). Among biomarkers, ferritin was almost always tested, while IL-18 (25%), S100 proteins (19%), and HLA-DRB1*15 (17%) were less frequently assessed. Before starting glucocorticoids (GCs) 68% of clinicians deemed certain investigations mandatory: bone marrow aspiration (74%), blood coltures and TB screening (50%), chest-abdomen CT scan and bone marrow biopsy (~20%). Beginning of steroid tapering was guided mostly by clinical and laboratory parameters: acute phase reactants (95%) and ferritin (87%) reduction, fever resolution (82%, ≥1 week: 37%; ≥72h: 29%; ≥2 weeks: 16%), arthritis improvement (75%), steroid side effects (42%); 29% and 25% requires normalization of acute phase reactants and ferritin, respectively, to start GCs tapering. More than half of physicians took patient/parent-reported outcomes (51% global VAS, 35% pain VAS) in consideration. Steroid discontinuation required at least 4-6 weeks of inactive disease for 40% of participants, and ≥3 months for 34%. Biologics withdrawal was considered after at least 6 months (35%), 1 year (35%) and 3 months (36%) of inactive disease. Factors driving biologics withdrawal were similar to ones influencing steroid tapering.

Conclusion: A considerable heterogeneity in clinical practice still exist, in particular intiming and factors guiding treatment tapering and withdrawal. Fostering harmonization of management of SD is essential to ensure consistent and optimal care for patients across different clinical settings

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; M. Jelusic: None; J. Anton: None; 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, 12, MAS Adjudication committee, American Board of Pediatrics, 12, Question writer for pediatric rheumatology boards, AS2 Biotherapeutics, 2, 12, Data Safety monitoring board, CareerPhysician, 1, 2, Neurogene, 2, Pfizer, 12, MAS Adjudication committee, Sobi, 1, 2, 5, Springer, 9, VIDA Ventures, 2; F. De Benedetti: AbbVie, 2, 5, Apollo, 2, 5, Elixiron, 2, 5, Kiniksa, 2, 5, Novartis, 2, 5, Sanofi, 2, 5, Sobi, 2, 5; A. Grom: Novartis, 2, 5, Sobi, 2, 5, UpToDate, 9; M. Heshin Bekenstein: None; A. Horne: None; R. Khubchandani: None; M. Mizuta: None; S. Özen: Novartis, 6, Pfizer, 6, Sobi, 6; P. Quartier Dit Maire: AbbVie/Abbott, 2, Amgen, 2, 5, Bristol-Myers Squibb(BMS), 2, 5, Chugai-Roche, 6, Lilly, 2, 5, Novartis, 2, 5, 6, Pfizer, 2, 5, 6, Sanofi, 2, 5, SOBI, 2, 5; 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; M. Shimizu: None; G. Schulert: IpiNovoyx, 5, Novartis, 2, SOBI, 2; C. Scott: None; R. Sinha: None; 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; J. Swart: 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; 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, Jelusic M, Anton J, Brogan P, Canna S, Cron R, De Benedetti F, Grom A, Heshin Bekenstein M, Horne A, Khubchandani R, Mizuta M, Özen S, Quartier Dit Maire P, Ravelli A, Shimizu M, Schulert G, Scott C, Sinha R, Ruperto N, Swart J, Fautrel B, Vastert S, Minoia F. Factors driving therapeutic decision-making in Still’s Disease: When to Start and When to Stop? Data from the METAPHOR Project Worldwide Survey [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/factors-driving-therapeutic-decision-making-in-stills-disease-when-to-start-and-when-to-stop-data-from-the-metaphor-project-worldwide-survey/. Accessed .

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