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. 2025 Jun 1;64(6):3587-3597.
doi: 10.1093/rheumatology/keaf057.

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

Chandni Sarker  1 , Andrea L Jorgensen  2 , Kukatharmini Tharmaratnam  2 , Eslam Al-Abadi  3 , Kate Armon  4 , Kathryn Bailey  5 , Marek Bohm  6 , Mary Brennan  7 , Coziana Ciurtin  8 , Janet Gardner-Medwin  9 , Daniel P Hawley  10 , Alison Kinder  11 , Alice Leahy  12 , Gulshan Malik  13 , Zoe McLaren  14 , Elena Moraitis  15 , Ellen Mosley  16 , Athimalaipet V Ramanan  17 , Satyapal Rangaraj  18 , Annie Ratcliffe  19 , Philip Riley  20 , Heather Rostron  6 , Ethan S Sen  21 , Christian M Hedrich  1   22 , Michael W Beresford  1   22 , Eve M D Smith  1   22
Affiliations

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

Chandni Sarker et al. Rheumatology (Oxford). .

Abstract

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.

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

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Figures

None
Graphical abstract
Figure 1.
Figure 1.
Venn diagrams depicting overlap between attainment of cSLE and aSLE targets. (A) Overlap between low disease activity targets (LLDAS and cLLDAS). (B) Overlap between remission targets (DORIS 2021 Remission and cCR). aSLE: adult-onset systemic lupus erythematosus; cCR: cSLE clinical remission on steroids; cCR-0: cSLE clinical remission off steroids; cLLDAS: childhood lupus low disease activity state; cSLE: childhood-onset systemic lupus erythematosus; DORIS 2021 Remission: single definition of remission proposed by the DORIS Task Force in 2021; LLDAS: lupus low disease activity state
Figure 2.
Figure 2.
Circular packing plots indicating number of visits for non-attainment of each criterion for each target. (A) Non-attainment of cLLDAS despite a SLEDAI-2K score of ≤4. (B) Non-attainment of LLDAS despite a SLEDAI-2K score of ≤4. (C) Non-attainment of cCR despite the cSLEDAI score being 0. (D) Non-attainment of DORIS 2021 Remission despite the cSLEDAI score being 0. (E) Non-attainment of cCR-0 despite the cSLEDAI score being 0. cCR: cSLE clinical remission on steroids; cCR-0: cSLE clinical remission off steroids; cLLDAS: childhood lupus low disease activity state; LLDAS: lupus low disease activity state; DORIS 2021 Remission: single definition of remission proposed by the DORIS Task Force in 2021; PGA: physician global assessment
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References

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