Practice Guideline

. 2025 Jul;84(7):1055-1067.

doi: 10.1016/j.ard.2025.04.024. Epub 2025 May 22.

Treating juvenile dermatomyositis to target: Paediatric Rheumatology European Society/Childhood Arthritis and Rheumatology Research Alliance-endorsed recommendations from an international task force

Angelo Ravelli¹, Silvia Rosina², Jayne M MacMahon³, Talia Baird⁴, Ana Isabel Rebollo-Giménez⁵, Claas Hinze⁶, Liza J McCann⁷, Ann M Reed⁸, Lisa G Rider⁹, Matilde Arvigo¹⁰, Brigitte Bader-Meunier¹¹, Claudio Bruno¹², Li Caifeng¹³, Raquel Campanilho-Marques¹⁴, Sara Cuccato¹⁰, Chiara Fiorillo¹⁵, Nikki A Hahn¹⁶, Adam M Huber¹⁷, Marc Jansen¹⁸, Ozgur Kasapcopur¹⁹, Maria Martha Katsikas²⁰, Susan Kim²¹, Polly Livermore²², Sue Maillard²³, Clara Malattia²⁴, Angela Nyangore Migowa²⁵, Takako Miyamae²⁶, Ruth Murphy²⁷, Rebecca Nicolai²⁸, Charalampia Papadopoulou²⁹, Clarissa Pilkington³⁰, Helga Sanner³¹, Sujata Sawhney³², Elzbieta Smolewska³³, Stacey E Tarvin³⁴, Georgina Tiller³⁵, Natasa Toplak³⁶, Lucy R Wedderburn³⁷, Francesca Bovis³⁸, Alessandro Consolaro²⁴, Brian M Feldman³⁹

Affiliations

¹ Direzione Scientifica, IRCCS Istituto Giannina Gaslini, Genoa, Italy; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy. Electronic address: angeloravelli@gaslini.org.
² UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
³ Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada; Child Health Evaluative Sciences (CHES), SickKids Research Institute, Toronto, Ontario, Canada.
⁴ Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
⁵ Department of Rheumatology, Hospital General Universitario Gregorio Marañón University Hospital, Gregorio Marañón Health Research Institute (IiSGM), Madrid, Spain.
⁶ Department of Pediatric Rheumatology and Immunology, University Hospital Münster, Münster, Germany.
⁷ Department of Paediatric Rheumatology, Institute in the Park, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom; Department of Women and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom.
⁸ Department of Pediatrics, Duke University, Durham, NC, USA.
⁹ Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA.
¹⁰ Patient representative.
¹¹ Department of Paediatric Hematology-Immunology and Rheumatology, Necker-Enfants Malades Hospital, AP-HP, Paris, France; National Reference Centre for Inflammatory Diseases and Pediatric Rheumatology - Immunology, Hematology and Pediatric Rheumatology Unit, Université Paris-Cité, IMAGINE Institute, Necker Children's Hospital, Paris, France.
¹² Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy; Division of Translational and Experimental Myology, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
¹³ Department of Rheumatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
¹⁴ Pediatric Rheumatology Unit, Pediatric and Rheumatology Department, Lisbon Academic Medical Center, Lisbon, Portugal.
¹⁵ Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy; Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.
¹⁶ Parent representative; Cure JM Foundation representative.
¹⁷ IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada.
¹⁸ Department of Paediatric Rheumatology and Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, Netherlands.
¹⁹ Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey.
²⁰ Servicio de Inmunología y Reumatología, Hospital Nacional de Pediatría Juan P. Garrahan, Buenos Aires, Argentina.
²¹ Division of Pediatric Rheumatology, Department of Pediatrics, University of California, San Francisco, CA, USA.
²² Rheumatology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
²³ Department of Paediatric Rheumatology, Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.
²⁴ Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy; UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
²⁵ Department of Paediatrics and Child Health, Aga Khan University Medical College East Africa, Nairobi, Kenya.
²⁶ Department of Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women's Medical University Hospital, Tokyo, Japan.
²⁷ University of Sheffield and Sheffield University Teaching Hospitals, Sheffield, United Kingdom.
²⁸ Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
²⁹ Paediatric Rheumatology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom; Inflammation and Rheumatology Section, University College London Great Ormond Street Institute of Child Health, London, United Kingdom.
³⁰ Blood, Cells and Cancer, Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.
³¹ Department of Rheumatology, Oslo University Hospital, Oslo, Norway; Department of Health Sciences, Oslo New University College, Oslo, Norway.
³² Division of Pediatric Rheumatology, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi, India.
³³ Department of Paediatric Cardiology and Rheumatology, Medical University of Lodz, Lodz, Poland.
³⁴ Division of Rheumatology, Riley Hospital for Children at Indiana University School of Medicine, Indianapolis, IN, USA.
³⁵ Division of Paediatric Rheumatology, The Royal Children's Hospital, Melbourne, Australia; Division of Paediatric Rheumatology, Monash Children's Hospital, Melbourne, Australia.
³⁶ Department of Allergology, Rheumatology and Clinical Immunology, UCH, UMC Ljubljana, Medical Faculty, Ljubljana, Slovenia.
³⁷ Infection, Immunity and Inflammation Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, London, United Kingdom; NIHR Biomedical Research Centre, Great Ormond Street Hospital, London, United Kingdom; Centre for Adolescent Rheumatology Versus Arthritis at UCL, UCL Hospital and Great Ormond Street Hospital, London, United Kingdom.
³⁸ Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy.
³⁹ Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Departments of Pediatrics, Medicine, Faculty of Medicine, and Institute of Health Policy Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada.

PMID: 40410052
PMCID: PMC12229752 (available on 2026-05-22)
DOI: 10.1016/j.ard.2025.04.024

Practice Guideline

Treating juvenile dermatomyositis to target: Paediatric Rheumatology European Society/Childhood Arthritis and Rheumatology Research Alliance-endorsed recommendations from an international task force

Angelo Ravelli et al. Ann Rheum Dis. 2025 Jul.

. 2025 Jul;84(7):1055-1067.

doi: 10.1016/j.ard.2025.04.024. Epub 2025 May 22.

Authors

Affiliations

¹ Direzione Scientifica, IRCCS Istituto Giannina Gaslini, Genoa, Italy; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy. Electronic address: angeloravelli@gaslini.org.
² UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
³ Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada; Child Health Evaluative Sciences (CHES), SickKids Research Institute, Toronto, Ontario, Canada.
⁴ Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
⁵ Department of Rheumatology, Hospital General Universitario Gregorio Marañón University Hospital, Gregorio Marañón Health Research Institute (IiSGM), Madrid, Spain.
⁶ Department of Pediatric Rheumatology and Immunology, University Hospital Münster, Münster, Germany.
⁷ Department of Paediatric Rheumatology, Institute in the Park, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom; Department of Women and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom.
⁸ Department of Pediatrics, Duke University, Durham, NC, USA.
⁹ Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA.
¹⁰ Patient representative.
¹¹ Department of Paediatric Hematology-Immunology and Rheumatology, Necker-Enfants Malades Hospital, AP-HP, Paris, France; National Reference Centre for Inflammatory Diseases and Pediatric Rheumatology - Immunology, Hematology and Pediatric Rheumatology Unit, Université Paris-Cité, IMAGINE Institute, Necker Children's Hospital, Paris, France.
¹² Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy; Division of Translational and Experimental Myology, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
¹³ Department of Rheumatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
¹⁴ Pediatric Rheumatology Unit, Pediatric and Rheumatology Department, Lisbon Academic Medical Center, Lisbon, Portugal.
¹⁵ Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy; Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.
¹⁶ Parent representative; Cure JM Foundation representative.
¹⁷ IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada.
¹⁸ Department of Paediatric Rheumatology and Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, Netherlands.
¹⁹ Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey.
²⁰ Servicio de Inmunología y Reumatología, Hospital Nacional de Pediatría Juan P. Garrahan, Buenos Aires, Argentina.
²¹ Division of Pediatric Rheumatology, Department of Pediatrics, University of California, San Francisco, CA, USA.
²² Rheumatology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
²³ Department of Paediatric Rheumatology, Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.
²⁴ Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova, Genoa, Italy; UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
²⁵ Department of Paediatrics and Child Health, Aga Khan University Medical College East Africa, Nairobi, Kenya.
²⁶ Department of Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women's Medical University Hospital, Tokyo, Japan.
²⁷ University of Sheffield and Sheffield University Teaching Hospitals, Sheffield, United Kingdom.
²⁸ Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
²⁹ Paediatric Rheumatology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom; Inflammation and Rheumatology Section, University College London Great Ormond Street Institute of Child Health, London, United Kingdom.
³⁰ Blood, Cells and Cancer, Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.
³¹ Department of Rheumatology, Oslo University Hospital, Oslo, Norway; Department of Health Sciences, Oslo New University College, Oslo, Norway.
³² Division of Pediatric Rheumatology, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi, India.
³³ Department of Paediatric Cardiology and Rheumatology, Medical University of Lodz, Lodz, Poland.
³⁴ Division of Rheumatology, Riley Hospital for Children at Indiana University School of Medicine, Indianapolis, IN, USA.
³⁵ Division of Paediatric Rheumatology, The Royal Children's Hospital, Melbourne, Australia; Division of Paediatric Rheumatology, Monash Children's Hospital, Melbourne, Australia.
³⁶ Department of Allergology, Rheumatology and Clinical Immunology, UCH, UMC Ljubljana, Medical Faculty, Ljubljana, Slovenia.
³⁷ Infection, Immunity and Inflammation Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, London, United Kingdom; NIHR Biomedical Research Centre, Great Ormond Street Hospital, London, United Kingdom; Centre for Adolescent Rheumatology Versus Arthritis at UCL, UCL Hospital and Great Ormond Street Hospital, London, United Kingdom.
³⁸ Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy.
³⁹ Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Departments of Pediatrics, Medicine, Faculty of Medicine, and Institute of Health Policy Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada.

PMID: 40410052
PMCID: PMC12229752 (available on 2026-05-22)
DOI: 10.1016/j.ard.2025.04.024

Abstract

Objectives: Despite the recent prognostic improvement, a sizeable proportion of patients with juvenile dermatomyositis (JDM) respond suboptimally to contemporary therapies. This study aimed to develop recommendations for treating JDM to target.

Methods: A Steering Committee formulated a set of provisional recommendations based on evidence derived from a systematic literature review and a retrospective chart review of patients. These were discussed, amended, and voted on by an international Task Force, including 28 paediatric rheumatologists, 2 specialists in neuromuscular diseases, 1 dermatologist, 1 physical therapist, 1 research nurse, 2 patients with JDM, and 1 parent of a patient with JDM. Items that achieved at least an 80% majority vote were accepted as final recommendations.

Results: Although the literature review did not reveal trials that compared a treat-to-target strategy with a nonsteered approach, it provided indirect evidence about specific end points that could serve as targets that facilitated development of recommendations. The group reached consensus on 7 overarching principles and 12 recommendations. It was agreed that both patients/parents and treaters should share decisions in setting treatment targets and therapeutic strategies, with inactive disease as the preferred target and minimal disease activity an alternative one. Inactive disease is targeted to be achieved within 12 months after treatment start. Interim targets include minimal and moderate clinical improvement within 6 weeks and 3 months, respectively, and normalisation of muscle strength within 6 months. High-dose glucocorticoids remain fundamental in the initial management, but progressive tapering and discontinuation within 12 months through optimisation of concomitant immunomodulatory therapy was advised. A research agenda was formulated.

Conclusions: The Task Force developed recommendations for treating JDM to target, being aware that the evidence is not strong and needs to be expanded by future research. Implementation of the recommendations in clinical practice will help to reach optimal outcomes for JDM.

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Conflict of interest statement

Competing interests All authors declare they have no competing interests related to the present work.

References

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Treating juvenile dermatomyositis to target: Paediatric Rheumatology European Society/Childhood Arthritis and Rheumatology Research Alliance-endorsed recommendations from an international task force

Affiliations

Treating juvenile dermatomyositis to target: Paediatric Rheumatology European Society/Childhood Arthritis and Rheumatology Research Alliance-endorsed recommendations from an international task force

Authors

Affiliations

Abstract

Conflict of interest statement

References

Publication types

MeSH terms

Substances

Grants and funding

LinkOut - more resources

Full Text Sources