PReS-endorsed international childhood lupus T2T task force definition of childhood lupus low disease activity state (cLLDAS)

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 Ozen; 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.2023.109296

PReS-endorsed international childhood lupus T2T task force definition of childhood lupus low disease activity state (cLLDAS)

Clin Immunol. 2023 May:250:109296. doi: 10.1016/j.clim.2023.109296. Epub 2023 Mar 17.

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 Ozen¹³, 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, S Marks²¹, L Oni²²

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 Department of Pediatric Rheumatology, Botucatu Medical School, Sao Paulo State University, 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 Clinical Research Centre, 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 Great Ormond Street Hospital for Children NHS Foundation Trust and the NIHR Great Ormond Street Hospital Biomedical Research Centre, London, UK.
²² Department of Women''s & Children''s Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool UK.

Abstract

Objective: To achieve a consensus-based definition of Low Disease Activity (LDA) for use in cSLE trials.

Methods: The International cSLE T2T Task Force, comprising of paediatric rheumatologists/nephrologists, and adult rheumatologists undertook a series of Delphi surveys/consensus meetings to discuss, refine, and vote upon cSLE LDA criteria.

Results: The Task Force agreed that LDA should be based upon the adult-SLE Lupus Low Disease Activity State definition (LLDAS), with modifications to make it applicable to cSLE (cLLDAS). They agreed upon five cLLDAS criteria: (1) SLE Disease Activity Index (SLEDAI)-2 K ≤4, with no activity in major organ systems; (2) no new features of lupus disease activity compared with the last assessment; (3) Physician Global Assessment score of ≤1 (0-3 scale); (4) prednisolone dose of ≤0.15 mg/kg/day, 7.5 mg/day/maximum; while on (5) stable antimalarials, immunosuppressives, and biologics.

Conclusions: A cSLE-appropriate definition of cLLDAS has been generated, maintaining alignment with the adult-SLE definition to promote life-course research.

Keywords: Childhood-SLE; Low disease activity; T2T; Treat-to-target; cLLDAS; cSLE.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Child
Consensus
Humans
Immunosuppressive Agents* / therapeutic use
Lupus Erythematosus, Systemic* / diagnosis
Lupus Erythematosus, Systemic* / drug therapy
Prednisolone
Severity of Illness Index

Substances

Immunosuppressive Agents
Prednisolone

Abstract

Publication types

MeSH terms

Substances

Grants and funding