Toggle Main Menu Toggle Search

Open Access padlockePrints

Validation of childhood lupus specific targets: Ensuring accurate assessment of disease control in younger, lighter paediatric patients

Lookup NU author(s): Dr Ethan SenORCiD

Downloads


Licence

This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


Abstract

© 2025 The Author(s).Objectives: To validate novel childhood-onset systemic lupus erythematosus (cSLE) treat-To-Target 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 (hazard ratio [HR] 0.18 [95% CI: 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, respectively) and lighter (median weight 26.8 and 37.1 kg, respectively) patients 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/day, respectively). Conclusions: This study validates novel paediatric-specific targets, demonstrating that achieving cLLDAS, cCR and cCR-0 reduces 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.


Publication metadata

Author(s): 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, Smith EMD

Publication type: Article

Publication status: Published

Journal: Rheumatology

Year: 2025

Volume: 64

Issue: 6

Pages: 3587-3597

Print publication date: 01/06/2025

Online publication date: 06/02/2025

Acceptance date: 23/01/2025

Date deposited: 09/06/2025

ISSN (print): 1462-0324

ISSN (electronic): 1462-0332

Publisher: Oxford University Press

URL: https://doi.org/10.1093/rheumatology/keaf057

DOI: 10.1093/rheumatology/keaf057

Data Access Statement: Data will be made available on reasonable request

PubMed id: 39913509


Altmetrics

Altmetrics provided by Altmetric


Funding

Funder referenceFunder name
Experimental Arthritis Treatment Centre for Children’ supported by Versus Arthritis (grant number ARUK-20621)
LUPUS UK: JXR10500, JXR12309]
Medical Research Foundation Emerging Lupus Leaders Prize

Share