EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
Por:
Fautrel B, Mitrovic S, De Matteis A, Bindoli S, Anton-Lopez J, Belot A, Bracaglia C, Constantin T, Dagna L, Di Bartolo A, Feist E, Foell D, Gattorno M, Georgin-Lavialle S, Giacomelli R, Grom AA, Jamilloux Y, Laskari K, Lazar C, Minoia F, Nigrovic PA, Oliveira Ramos F, Ozen S, Quartier P, Ruscitti P, Sag E, Savic S, Truchetet ME, Vastert SJ, Wilhelmer TC, Wouters C, Carmona L and De Benedetti F
Publicada:
1 dic 2024
Ahead of Print:
1 sep 2024
Resumen:
Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing.
Methods In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS). Systematic literature reviews were conducted accordingly.
Results The TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Still's disease. Fourteen specific recommendations were issued. Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). MAS treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-gamma inhibitors. A specific concern rose recently with cases of severe lung disease in children with Still's disease, for which T cell directed immunosuppressant are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients. All overarching principles and recommendations were agreed by over 80% of the TF experts with a high level of agreement.
Conclusion These recommendations are the first consensus for the diagnosis and management of children and adults with Still's disease.
Filiaciones:
Fautrel B:
Rheumatology, CEREMAIA Reference Center (ERN RITA) , Sorbonne Université - AP-HP, Pitié-Salpêtrière Hospital, Paris, France
INSERM UMR-S 1136, Team 5, Pierre Louis Institute of Epidemiology and Public Health, Paris, France
CRI-IMIDIATE Clinical Research Network, Assistance Publique - Hopitaux de Paris, Paris, France
Mitrovic S:
Rheumatology, CEREMAIA Reference Center (ERN RITA) , Sorbonne Université - AP-HP, Pitié-Salpêtrière Hospital, Paris, France
De Matteis A:
Division of Rheumatology, ERN RITA center, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Bindoli S:
Rheumatology, Università degli Studi di Padova, Padova, Veneto, Italy
Anton-Lopez J:
Department of Pediatric Rheumatology, Hospital Sant Joan de Deu, Universitat de Barcelona, Barcelona, Spain
CSUR Enfermedades Autoinmunes y Autoinflamatorias, ERN RITA, Barcelona, Spain
Belot A:
Department of Paediatric Nephrology, Rheumatology, Dermatology, Hôpital Femme-Mère Enfant, Université Claude Bernard-Lyon 1, Bron, France
Centre International de Recherche en Infectiologie (CIRI), Inserm UMR 1111, CNRS, UMR5308, Ecole Normale Supérieure de Lyon, Lyon, France
National Reference Centres for Rheumatism & AutoImmune diseaSes in children (RAISE) and Autoinflammatory diseases & Amyloidosis (CEREMAIA), ERN RITA RECONNECT, Lyon, France
Bracaglia C:
Division of Rheumatology, ERN RITA center, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Constantin T:
Unit of Paediatric Rheumatology, Semmelweis University, Budapest, Hungary
Dagna L:
Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milano, Italy
Di Bartolo A:
PReS Patient Research Partner, Rome, Italy
Feist E:
Rheumatology and Clinical Immunology, HELIOS Fachklinik Vogelsang/Gommern, Vogelsang, Sachsen-Anhalt, Germany
Foell D:
Department of Rheumatology and Clinical Immunology, Children Hospital, University of Muenster, Muenster, Germany
Gattorno M:
UOSD Centro Malattie Autoinfiammatorie e Immunodeficienze, IRCCS Istituto Giannina Gaslini, Genoa, Italy
Georgin-Lavialle S:
Internal Medicine, CEREMAIA Reference Center (ERN RITA), Tenon Hospital , Sorbonne Université, AP-HP, Paris, France
Giacomelli R:
Università Campus Bio-Medico di Roma Facoltà Dipartimentale di Medicina e Chirurgia, Roma, Italy
Grom AA:
Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA
Jamilloux Y:
Internal Medicine, CEREMAIA Reference Center (ERN RITA), Croix Rousse Hospital, Hospices Civils de Lyon - Université Claude Bernard - Lyon 1, Lyon, France
Laskari K:
Rheumatology Unit, 1st Dept. of Propaedeutic Internal Medicine, National & Kapodistrian University of Athens Medical School, Athens, Greece
Lazar C:
Department of Pediatric Rheumatology, University of Medicine and Pharmacy Iuliu Hatieganu, Cluj-Napocca, Romania
Minoia F:
Pediatria e Immunoreumatologia, IRCCS Foundation Maggiore Policlinico Hospital, Milan, Italy
Nigrovic PA:
Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Boston, MA, USA
Immunology, Boston Children's Hospital, Harvard University, Boston, MA, USA
Oliveira Ramos F:
Unidade de Reumatologia Pediátrica, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Center, Lisbon, Portugal
Ozen S:
Pediatric Rheumatology, Hacettepe University, Ankara, Turkey
Quartier P:
Pediatric Immuno-Hematology and Rheumatology Unit, RAISE Reference Center (ERN RECONNECT), Hopital universitaire Necker-Enfants malades, Paris, France
IMAGINE Institute, Université de Paris, Assistance Publique-Hopitaux de Paris, Paris, France
Ruscitti P:
Division of Rheumatology, Department of Clinical Sciences and Applied Biotechnology, University of L'Aquila, L'Aquila, Abruzzo, Italy
Sag E:
Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University, Ankara, Turkey
Savic S:
Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK
Clinical Immunology and Allergy, NIHR-Leeds Biomedical research centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Truchetet ME:
Rhumatologie, CHU Bordeaux, Bordeaux, France
ImmunoConcEpT, UMR 5164, Université de Bordeaux, CNRS, Bordeaux, France
Centre de Référence Maladies Autoimmunes et Systémiques rares Est / Sud Ouest (RESO), RECONNECT ERN, FHU ACRONIM, CHU Bordeaux, Bordeaux, France
Vastert SJ:
Pediatric Immunology, University Medical Center Utrecht, Wilhelmina Children's Hospital, Utrecht, The Netherlands
Wilhelmer TC:
EULAR Young PARE, Zürich, Switzerland
Österreichische Rheumaliga, Maria Alm, Austria
Wouters C:
Pediatric Immunology, University Hospital Gasthuisberg, Leuven, Belgium
Department of Pediatric Rheumatology, Centre for Rare Immune Deficiency, Autoimmune and Autoinflammatory Diseases, UZ Leuven, Leuven, Belgium
Carmona L:
Instituto de Salud Musculoesquelética (INMUSC), Madrid, Madrid, Spain
De Benedetti F:
Division of Rheumatology, ERN RITA center, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Green Submitted, hybrid
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