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Rheumatology (Oxford). 2021 Dec 11; doi: 10.1093/rheumatology/keab915. Epub 2021 Dec 11.

Attainment of Low Disease Activity and Remission Targets reduces the risk of severe flare and new damage in Childhood Lupus.

Rheumatology (Oxford, England)

Eve M D Smith, Kukatharmini Tharmaratnam, Eslam Al-Abadi, Kate Armon, Kathryn Bailey, Mary Brennan, Coziana Ciurtin, Janet Gardner-Medwin, Kirsty E Haslam, Daniel Hawley, Alice Leahy, Valentina Leone, Gulshan Malik, Zoe McLaren, Clarissa Pilkington, Athimalaipet V Ramanan, Satyapal Rangaraj, Annie Ratcliffe, Philip Riley, Ethan Sen, Arani Sridhar, Nick Wilkinson, Christian M Hedrich, Andrea Jorgensen, Michael W Beresford

Affiliations

  1. Department of Women's & Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK.
  2. Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK.
  3. Department of Health Data Science, Institute of Population Health, University of Liverpool, Liverpool, UK.
  4. Department of Rheumatology, Birmingham Children's Hospital, Birmingham, UK.
  5. Department of Paediatric Rheumatology, Cambridge University Hospitals, Cambridge, UK.
  6. Department of Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
  7. Department of Paediatric Rheumatology, Royal Hospital for Sick Children, Edinburgh, UK.
  8. Centre for Adolescent Rheumatology, University College London, London, UK.
  9. Department of Child Health, University of Glasgow, Glasgow, UK.
  10. Department of Paediatrics, Bradford Royal Infirmary, Bradford, UK.
  11. Department of Paediatric Rheumatology, Sheffield Children's Hospital, Sheffield, UK.
  12. Department of Paediatric Rheumatology, Southampton General Hospital, Southampton, UK.
  13. Department of Paediatric Rheumatology, Leeds Children Hospital, Leeds, UK.
  14. Paediatric Rheumatology, Royal Aberdeen Children's Hospital, Aberdeen, UK.
  15. Rheumatology Department, Aintree University Hospital, Liverpool, UK.
  16. Department of Paediatric Rheumatology, Great Ormond Street Hospital, London, UK.
  17. University Hospitals Bristol NHS Foundation Trust & Bristol Medical School, University of Bristol, Bristol, UK.
  18. Department of Paediatric Rheumatology, Nottingham University Hospitals, Nottingham, UK.
  19. Department of Paediatrics, Musgrove Park Hospital, Taunton, UK.
  20. Department of Paediatric Rheumatology, Royal Manchester Children's Hospital, Manchester, UK.
  21. Paediatric Rheumatology, Great North Children's Hospital & Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK.
  22. Leicester Children's Hospital, University Hospitals of Leicester NHS trust, Leicester, UK.
  23. Guy's & St Thomas's NHS Foundation Trust, Evelina Children's Hospital, London, UK.

PMID: 34894234 DOI: 10.1093/rheumatology/keab915

Abstract

OBJECTIVES: To assess the achievability and effect of attaining low disease activity (LDA) or remission in childhood (cSLE).

METHODS: Attainment of three adult-SLE derived definitions of LDA (LLDAS, LA, Toronto-LDA), and four definitions of remission (clinical-SLEDAI-defined remission on/off treatment, pBILAG-defined remission on/off treatment) was assessed in UK JSLE Cohort Study patients longitudinally. Prentice-Williams-Petersen-GAP recurrent event models assessed the impact of LDA/remission attainment on severe flare/new damage.

RESULTS: LLDAS, LA and Toronto-LDA targets were reached in 67%, 73% and 32% of patients, after a median of 18, 15 or 17 months, respectively. Cumulatively, LLDAS, LA and Toronto-LDA was attained for a median of 23%, 31% and 19% of total follow-up-time, respectively. Remission on-treatment was more common (61% cSLEDAI-defined, 42% pBILAG-defined) than remission off-treatment (31% cSLEDAI-defined, 21% pBILAG-defined). Attainment of all target states, and disease duration (>1 year), significantly reduced the hazard of severe flare (p< 0.001). As cumulative time in each target increased, hazard of severe flare progressively reduced. LLDAS attainment reduced the hazard of severe flare more than LA or Toronto-LDA (p< 0.001). Attainment of LLDAS and all remission definitions led to a statistically comparable reduction in the hazards of severe flare (p> 0.05). Attainment of all targets reduced the hazards of new damage (p< 0.05).

CONCLUSIONS: This is the first study demonstrating that adult-SLE-derived definitions of LDA/remission are achievable in cSLE, significantly reducing risk of severe flare/new damage. Of the LDA definitions, LLDAS performed best, leading to a statistically comparable reduction in the hazards of severe flare to attainment of clinical-remission.

© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology.

Keywords: Childhood-SLE; T2T; cSLE; low disease activity; remission; treat-to-target

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