Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

E M D Smith; A Aggarwal; J Ainsworth; E Al-Abadi; T Avcin; L Bortey; J Burnham; C Ciurtin; C M Hedrich; S Kamphuis; L Lambert; D M Levy; L Lewandowski; N Maxwell; E Morand; S Özen; C E Pain; A Ravelli; C Saad Magalhaes; C Pilkington; D Schonenberg-Meinema; C Scott; K Tullus; M W Beresford; International cSLE T2T Task Force

doi:10.1016/j.clim.2024.110214

Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

Clin Immunol. 2024 Jun:263:110214. doi: 10.1016/j.clim.2024.110214. Epub 2024 Apr 9.

Authors

E M D Smith¹, A Aggarwal², J Ainsworth³, E Al-Abadi⁴, T Avcin⁵, L Bortey⁶, J Burnham⁷, C Ciurtin⁸, C M Hedrich³, S Kamphuis⁹, L Lambert⁶, D M Levy¹⁰, L Lewandowski¹¹, N Maxwell⁶, E Morand¹², S Özen¹³, C E Pain³, A Ravelli¹⁴, C Saad Magalhaes¹⁵, C Pilkington¹⁶, D Schonenberg-Meinema¹⁷, C Scott¹⁸, K Tullus¹⁹, M W Beresford³; International cSLE T2T Task Force

Collaborators

International cSLE T2T Task Force:
B Goilav²⁰, N Goss²¹, L Oni²², S D Marks²³

Affiliations

¹ Department of Women's & Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK; Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK. Electronic address: esmith8@liverpool.ac.uk.
² Department of Clinical Immunology & Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
³ Department of Women's & Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK; Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
⁴ Department of Paediatric Rheumatology, Birmingham Women's and Children's Hospital NHS Foundation Trust, Birmingham, UK.
⁵ Department of Allergology, Rheumatology and Clinical Immunology, Children's Hospital, University Medical Center Ljubljana, Slovenia.
⁶ TARGET Lupus Public Patient Involvement and Engagement Group, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK.
⁷ Department of Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, USA.
⁸ Centre for Adolescent Rheumatology Versus Arthritis, Division of Medicine, University College London, UK.
⁹ Department of Pediatric Rheumatology, Sophia Children's Hospital, Erasmus University Medical Center, Rotterdam, Netherlands.
¹⁰ Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children and University of Toronto, Toronto, Canada.
¹¹ Lupus Genomics and Global Health Disparities Unit, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
¹² Centre for Inflammatory Diseases, Monash University, Melbourne, Australia.
¹³ Department of Pediatrics, Hacettepe University, Ankara, Turkey.
¹⁴ Direzione Scientifica, IRCCS Istituto Giannina Gaslini, Genoa, Italy; Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno Infantili (DINOGMI), Università degli Studi di Genova, Genoa, Italy.
¹⁵ Paediatric Rheumatology Division, Botucatu Medical School, Sao Paulo State, University (UNESP), Sao Paulo, Brazil.
¹⁶ Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
¹⁷ Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, the Netherlands.
¹⁸ Department of Pediatric Rheumatology, University of Cape Town, Cape Town, South Africa.
¹⁹ Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
²⁰ Albert Einstein College of Medicine, New York, USA.
²¹ Health and Life Sciences, University of Liverpool, Liverpool, UK.
²² Department of Paediatric Nephrology, Alder Hey children's NHS Foundation Trust, Department of Women's & Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK.
²³ Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; NIHR Great Ormond Street Hospital Biomedical Research Centre, University College London Great Ormond Street Institute of Child Health, London, UK.

PMID: 38604255
DOI: 10.1016/j.clim.2024.110214

Abstract

Objective: To derive childhood-onset SLE (cSLE) specific remission definitions for future treat-to-target (T2T) trials, observational studies, and clinical practice.

Methods: The cSLE International T2T Task Force conducted Delphi surveys exploring paediatric perspectives on adult-onset SLE remission targets. A modified nominal group technique was used to discuss, refine, and agree on the cSLE remission target criteria.

Results: The Task Force proposed two definitions of remission: 'cSLE clinical remission on steroids (cCR)' and 'cSLE clinical remission off steroids (cCR-0)'. The common criteria are: (1) Clinical-SLEDAI-2 K = 0; (2) PGA score < 0.5 (0-3 scale); (4) stable antimalarials, immunosuppressive, and biologic therapy (changes due to side-effects, adherence, weight, or when building up to target dose allowed). Criterion (3) in cCR is the prednisolone dose ≤0.1 mg/kg/day (maximum 5 mg/day), whereas in cCR-0 it is zero.

Conclusions: cSLE definitions of remission have been proposed, maintaining sufficient alignment with the adult-SLE definition to facilitate life-course research.

Keywords: Childhood-onset SLE; Remission; T2T; Treat-to-target; cSLE.

MeSH terms

Advisory Committees
Age of Onset
Child
Consensus*
Delphi Technique
Humans
Immunosuppressive Agents / therapeutic use
Lupus Erythematosus, Systemic* / diagnosis
Lupus Erythematosus, Systemic* / drug therapy
Remission Induction*