Validation of childhood lupus specific targets: ensuring accurate assessment of disease control in younger, lighter paediatric patients.
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Authors
Sarker, Chandni
Jorgensen, Andrea L
Tharmaratnam, Kukatharmini
Al-Abadi, Eslam
Armon, Kate
Bailey, Kathryn
Bohm, Marek
Brennan, Mary
Ciurtin, Coziana
Gardner-Medwin, Janet
Issue Date
2025-02-06
Type
Journal Article
Language
en
Keywords
Childhood-SLE , T2T , cSLE , low disease activity , remission , treat-to-target , Wessex Classification Subject Headings::Paediatrics , Wessex Classification Subject Headings::Diseases & disorders of systemic, metabolic or environmental origin::Rheumatology
Alternative Title
Abstract
Abstract
Objectives: To validate novel childhood-onset systemic lupus erythematosus (cSLE) T2T targets including Childhood Lupus Low Disease Activity State (cLLDAS), cSLE Clinical Remission on steroids (cCR), and cSLE Clinical Remission off steroids (cCR-0), as compared with adult-onset SLE (aSLE) targets.
Methods: Attainment of the aforementioned cSLE-specific and aSLE-specific targets (LLDAS, DORIS 2021 Remission) was assessed at each visit, in UK JSLE Cohort Study patients. Univariable and multivariable Prentice-Williams-Peterson (PWP) gap-time models investigated the impact of target attainment on new damage and severe flare.
Results: The cohort included 430 cSLE patients. Attainability was comparable between corresponding cSLE and aSLE targets. Achieving cLLDAS (HR 0.18 [0.14, 0.23]), cCR (HR 0.18 [0.13, 0.23]) and cCR-0 (HR 0.17 [0.13, 0.23]) reduced the risk of severe flare (all p < 0.001). Risk of new damage was reduced in those reaching cLLDAS (HR 0.22 [0.11, 0.44]), cCR (HR 0.25 [0.13, 0.49]) and cCR-0 (HR 0.30 [0.15, 0.60]) (all p < 0.001). Inappropriate attainment of LLDAS and DORIS remission occurred at 35 and 52 visits, respectively, in younger (median age 7.3 and 8.8 years) and lighter patients (median weight 26.8 and 37.1 kg) whilst on prednisolone doses that precluded cSLE target attainment (median 0.17 [IQR 0.16-0.24] and 0.13 [IQR 0.11-0.16] mg/kg respectively).
Conclusions: This study validates novel paediatric-specific targets, demonstrating that achieving cLLDAS, cCR, and cCR-0 reduce risks of new damage and severe flare, which is comparable to aSLE targets. Using cSLE-specific targets prevents misclassification of disease activity in paediatric patients, enabling more accurate disease control assessments in younger, lighter patients.
Description
Citation
Sarker C, Jorgensen AL, Tharmaratnam K, Al-Abadi E, Armon K, Bailey K, Bohm M, Brennan M, Ciurtin C, Gardner-Medwin J, Hawley DP, Kinder A, Leahy A, Malik G, McLaren Z, Moraitis E, Mosley E, Ramanan AV, Rangaraj S, Ratcliffe A, Riley P, Rostron H, Sen ES, Hedrich CM, Beresford MW, Md Smith E. Validation of childhood lupus specific targets: ensuring accurate assessment of disease control in younger, lighter paediatric patients. Rheumatology (Oxford). 2025 Feb 6:keaf057. doi: 10.1093/rheumatology/keaf057. Epub ahead of print. PMID: 39913509.
Publisher
Oxford Academic
License
© The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology.
Journal
Rheumatology (Oxford, England)
Volume
Issue
PubMed ID
ISSN
1462-0332