Development of CARRA/PReS-endorsed consensus Core and Expanded Datasets in childhood-onset systemic lupus erythematosus for international registry-based research

Rebecca E Sadun¹, Jennifer C Cooper², Alexandre Belot³, Tadej Avcin⁴, Amita Aggarwal⁵, Jenny Ainsworth⁶, Alisha Akinsete⁷, Stacy P Ardoin⁸, Michael W Beresford⁶, Lynette Bortey⁹, Hermine I Brunner¹⁰, Joyce C Chang¹¹, Coziana Ciurtin¹², Ashley Daftary¹³, Barbara Eberhard¹⁴, Candace H Feldman¹⁵, Christian M Hedrich⁶, Aimee O Hersh¹⁶, Linda T Hiraki¹⁷, David A Isenberg¹², Sylvia Kamphuis¹⁸, Andrea M Knight¹⁷, Lou Lambert⁹, Deborah M Levy¹⁷, Stephen D Marks¹⁹, Naomi Maxwell⁹, Angela Migowa²⁰, Katharine Moore², Seza Ozen²¹, Rosalind Ramsey-Goldman²², Angelo Ravelli²³, Bryce B Reeve²⁴, Tamar B Rubinstein²⁵, Claudia Saad-Magalhaes²⁶, Sujata Sawhney²⁷, Laura E Schanberg²⁸, Emily von Scheven²⁹, Christiaan Scott³⁰, Mary Beth Son¹¹, Gladys Tony³¹, Elissa R Weitzman³², Scott E Wenderfer³³, Alisha Woodside³¹, Laura B Lewandowski³⁴, Eve Md Smith³⁵; CARRA SLE Committee and PReS Lupus Working Party

Affiliations

¹ Departments of Medicine and Pediatrics, Duke University School of Medicine, Durham, North Carolina, USA.
² Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
³ Service de Pediatrie, Centre Hospitalier Universitaire de Lyon, Lyon, France. Electronic address: https://x.com/AlexandreBelot.
⁴ Department of Allergology, Rheumatology and Clinical Immunology, University Medical Centre, Ljubljana, Slovenia.
⁵ Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
⁶ Department of Women's & Children's Health, University of Liverpool, Liverpool, UK; Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
⁷ Department of Pediatrics, Hospital for Special Surgery, New York, New York, USA.
⁸ Departments of Medicine and Pediatrics, The Ohio State University College of Medicine, Columbus, Ohio, USA.
⁹ TARGET LUPUS PPIE Group, University of Liverpool, Liverpool, UK.
¹⁰ Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio, USA.
¹¹ Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
¹² Centre for Rheumatology, University College London, London, UK.
¹³ University of Nevada Reno School of Social Work, Reno, Nevada, USA.
¹⁴ Department of Pediatrics, Hofstra Northwell School of Medicine at Hofstra University, Hempstead, New York, USA.
¹⁵ Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, Boston, Massachusetts, USA.
¹⁶ The University of Utah School of Medicine, Salt Lake City, Utah, USA.
¹⁷ Division of Rheumatology and Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada; Division of Rheumatology, University of Toronto Temerty Faculty of Medicine, Toronto, Ontario, Canada.
¹⁸ Erasmus MC University Medical Center, Rotterdam, The Netherlands.
¹⁹ NIHR Great Ormond Street Hospital Biomedical Research Centre, University College London, London, UK; Institute of Child Health and Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
²⁰ Department of Pediatrics, The Aga Khan University Medical College East Africa, Nairobi, Kenya.
²¹ Department of Pediatrics, Hacettepe University, Ankara, Turkey.
²² Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
²³ Direzione Scientifica, IRCCS Istituto Giannina Gaslini and Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili, Università degli Studi di Genova, Genova, Italy.
²⁴ Departments of Population Health Sciences and Pediatrics, Duke University School of Medicine, Durham, North Carolina, USA.
²⁵ Children's Hospital at Montefiore, New York, New York, USA.
²⁶ Pediatric Rheumatology Division, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, Brazil.
²⁷ Institute of Child Health, Paediatric Rheumatology Division, Sir Ganga Ram Hospital, New Delhi, India.
²⁸ Department of Pediatrics, Duke University School of Medicine, Durham, North Carolina, USA.
²⁹ Department of Pediatrics, University of California San Francisco, San Francisco, California, USA.
³⁰ Department of Child and Adolescent Health, University of Cape Town, Rondebosch, South Africa; Division of Pediatric Rheumatology, University of Ottawa, Ottawa, Ontario, Canada.
³¹ Patient/Caregiver Representative, Childhood Arthritis and Rheumatology Research Alliance, Milwaukee, Wisconsin, USA.
³² Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA. Electronic address: https://x.com/Elissa_Weitzman.
³³ Department of Pediatrics, The University of British Columbia, Vancouver, Columbia, Canada; BC Children's Hospital, Vancouver, Columbia, Canada. Electronic address: https://x.com/PedsLupusRenal.
³⁴ National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Maryland, USA.
³⁵ Department of Women's & Children's Health, University of Liverpool, Liverpool, UK; Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK. Electronic address: esmith8@liverpool.ac.uk.

PMID: 39919891
PMCID: PMC12345348 (available on 2026-01-02)
DOI: 10.1136/ard-2024-226528

Development of CARRA/PReS-endorsed consensus Core and Expanded Datasets in childhood-onset systemic lupus erythematosus for international registry-based research

Rebecca E Sadun et al. Ann Rheum Dis. 2025 Feb.

. 2025 Feb;84(2):158-168.

doi: 10.1136/ard-2024-226528. Epub 2025 Jan 2.

Authors

Affiliations

¹ Departments of Medicine and Pediatrics, Duke University School of Medicine, Durham, North Carolina, USA.
² Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
³ Service de Pediatrie, Centre Hospitalier Universitaire de Lyon, Lyon, France. Electronic address: https://x.com/AlexandreBelot.
⁴ Department of Allergology, Rheumatology and Clinical Immunology, University Medical Centre, Ljubljana, Slovenia.
⁵ Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
⁶ Department of Women's & Children's Health, University of Liverpool, Liverpool, UK; Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
⁷ Department of Pediatrics, Hospital for Special Surgery, New York, New York, USA.
⁸ Departments of Medicine and Pediatrics, The Ohio State University College of Medicine, Columbus, Ohio, USA.
⁹ TARGET LUPUS PPIE Group, University of Liverpool, Liverpool, UK.
¹⁰ Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio, USA.
¹¹ Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
¹² Centre for Rheumatology, University College London, London, UK.
¹³ University of Nevada Reno School of Social Work, Reno, Nevada, USA.
¹⁴ Department of Pediatrics, Hofstra Northwell School of Medicine at Hofstra University, Hempstead, New York, USA.
¹⁵ Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, Boston, Massachusetts, USA.
¹⁶ The University of Utah School of Medicine, Salt Lake City, Utah, USA.
¹⁷ Division of Rheumatology and Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada; Division of Rheumatology, University of Toronto Temerty Faculty of Medicine, Toronto, Ontario, Canada.
¹⁸ Erasmus MC University Medical Center, Rotterdam, The Netherlands.
¹⁹ NIHR Great Ormond Street Hospital Biomedical Research Centre, University College London, London, UK; Institute of Child Health and Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
²⁰ Department of Pediatrics, The Aga Khan University Medical College East Africa, Nairobi, Kenya.
²¹ Department of Pediatrics, Hacettepe University, Ankara, Turkey.
²² Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
²³ Direzione Scientifica, IRCCS Istituto Giannina Gaslini and Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili, Università degli Studi di Genova, Genova, Italy.
²⁴ Departments of Population Health Sciences and Pediatrics, Duke University School of Medicine, Durham, North Carolina, USA.
²⁵ Children's Hospital at Montefiore, New York, New York, USA.
²⁶ Pediatric Rheumatology Division, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, Brazil.
²⁷ Institute of Child Health, Paediatric Rheumatology Division, Sir Ganga Ram Hospital, New Delhi, India.
²⁸ Department of Pediatrics, Duke University School of Medicine, Durham, North Carolina, USA.
²⁹ Department of Pediatrics, University of California San Francisco, San Francisco, California, USA.
³⁰ Department of Child and Adolescent Health, University of Cape Town, Rondebosch, South Africa; Division of Pediatric Rheumatology, University of Ottawa, Ottawa, Ontario, Canada.
³¹ Patient/Caregiver Representative, Childhood Arthritis and Rheumatology Research Alliance, Milwaukee, Wisconsin, USA.
³² Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA. Electronic address: https://x.com/Elissa_Weitzman.
³³ Department of Pediatrics, The University of British Columbia, Vancouver, Columbia, Canada; BC Children's Hospital, Vancouver, Columbia, Canada. Electronic address: https://x.com/PedsLupusRenal.
³⁴ National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Maryland, USA.
³⁵ Department of Women's & Children's Health, University of Liverpool, Liverpool, UK; Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK. Electronic address: esmith8@liverpool.ac.uk.

PMID: 39919891
PMCID: PMC12345348 (available on 2026-01-02)
DOI: 10.1136/ard-2024-226528

Abstract

Objectives: Childhood-onset systemic lupus erythematosus (cSLE), representing 15%-20% of individuals with SLE, has been difficult to study globally due to differences between registries. This initiative, supported by Childhood Arthritis Rheumatology Research Alliance (CARRA) and Paediatric Rheumatology European Society (PReS), aims to create Core and Expanded cSLE Datasets to standardise and enhance research worldwide.

Methods: 21 international cSLE experts and 4 patients participated in a Delphi process (questionnaires, 2 topic-specific focus groups and 3 virtual consensus meetings) to create 2 standardised cSLE datasets. The Core cSLE Dataset was designed to include data essential to meaningful clinical research across many settings. The Expanded cSLE Dataset was designed for centres able to consistently collect data to address broader research questions. Final data items for the Core and Expanded datasets were determined by consensus defined as >80% agreement) using an adapted nominal group technique and voting.

Results: The resulting Core cSLE Dataset contains 46 items, including demographics, clinical features, laboratory results, medications and significant adverse events. The Expanded cSLE Dataset adds 26 additional items and includes patient-reported outcomes. Consensus was also achieved regarding the frequency and time points for data collection: baseline, quarterly follow-up visits, annually and flare visits.

Conclusion: Standardised Core and Expanded cSLE Datasets for registry-based international cSLE research were defined through the consensus of global experts and patient/caregiver representatives, endorsed by CARRA and PReS. These datasets incorporate disease-specific and patient-specific features, optimised for diverse settings to facilitate international collaborative research for children and adolescents with SLE worldwide.

Keywords: Child; Health Care; Lupus Erythematosus; Lupus Nephritis; Outcome Assessment; Patient Reported Outcome Measures; Systemic.

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References

1. Bader-Meunier B, Armengaud JB, Haddad E, et al. Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study. J Pediatr 2005;146:648–53. doi: 10.1016/j.jpeds.2004.12.045. - DOI - PubMed
1. Smitherman EA, Chahine RA, Beukelman T, et al. Childhood-Onset Lupus Nephritis in the Childhood Arthritis and Rheumatology Research Alliance Registry: Short-Term Kidney Status and Variation in Care. Arthritis Care Res (Hoboken) 2023;75:1553–62. doi: 10.1002/acr.25002. - DOI - PMC - PubMed
1. Watson L, Leone V, Pilkington C, et al. Disease activity, severity, and damage in the UK juvenile–onset systemic lupus erythematosus cohort. Arthritis & Rheumatism 2012;64:2356–65. doi: 10.1002/art.34410. - DOI - PubMed
1. Brunner HI, Gladman DD, Ibañez D, et al. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum 2008;58:556–62. doi: 10.1002/art.23204. - DOI - PubMed
1. Hersh AO, von Scheven E, Yazdany J, et al. Differences in long-term disease activity and treatment of adult patients with childhood- and adult-onset systemic lupus erythematosus. Arthritis Rheum 2009;61:13–20. doi: 10.1002/art.24091. - DOI - PMC - PubMed

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Development of CARRA/PReS-endorsed consensus Core and Expanded Datasets in childhood-onset systemic lupus erythematosus for international registry-based research

Affiliations

Development of CARRA/PReS-endorsed consensus Core and Expanded Datasets in childhood-onset systemic lupus erythematosus for international registry-based research

Authors

Affiliations

Abstract

References

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Medical