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Validation of childhood lupus low disease activity state definition
Childhood-onset systemic lupus erythematosus (cSLE) has a more severe disease course compared to adult-onset SLE.1 The Lupus Low Disease Activity State (LLDAS) seems to be realistic treat-to-target approach in these patients.1 An international taskforce developed an adapted LLDAS for cSLE called childhood Lupus Low Disease Activity State (cLLDAS) due to an unmet need for a cSLE-specific definition in this population.2
Bergkamp et al.1 recently published an article in Clinical Immunology presenting the first study to validate the new cLLDAS definition in a real-life cohort of cSLE patients.1 Here, we summarize the key findings.
Methods1
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The new cLLDAS definition was retrospectively applied to prospectively collected data from the longitudinal, real-life cSLE cohort.
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The objectives of the study were to:
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compare the time to achieve first adult LLDAS (aLLDAS) vs cLLDAS;
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Assess if patients with cSLE could maintain cLLDAS for ≥50% of follow-up time (cLLDAS-50); and
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assess the predictors for cLLDAS-50 and occurrence of damage using univariate logistic regression analysis.
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Key findings1
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A total of 50 patients with cSLE were included; 86% were females and mean age at diagnosis was 14 years.
cLLDAS
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Each patient attained aLLDAS and cLLDAS at least once during their follow-up.
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Mean time to attain first aLLDAS and cLLDAS was 8.2 and 9.0 months, respectively.
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However, this difference was only due to one patient.
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In 22 of the 42 patients, the mean delay to achieve first cLLDAS due to steroid dosage was 6.2 months.
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Median time spent in first cLLDAS was 3 months, with 78% patients not remaining in first cLLDAS.
cLLDAS-50
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cLLDAS-50 was maintained in 58% of patients.
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The predictors for attaining cLLDAS-50, as per univariate analysis, are depicted in Figure 1A.
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In the multivariate analysis, a longer time from diagnosis to first cLLDAS (Odds ratio [OR], 0.828; p = 0.030) and higher number of flares (OR, 0.305; p = 0.04) were negative predictors for attaining cLLDAS-50.
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Damage
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Damage occurred in 34% of patients with a median follow-up of 3.1 years from diagnosis, with 64.7% experiencing damage within ≤1 year after diagnosis.
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The predictors of damage, as per univariate analysis, are depicted in Figure 1B.
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The significance of these predictors for damage disappeared in the multivariate model.
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Figure 1. Univariate logistic regression analysis of variables associated with A attainment of cLLDAS-50 and B occurrence of damage*
CI, confidence interval; cLLDAS, childhood lupus low disease activity state; OR, odds ratio; PGA, physician global assessment; SLEDAI, Systemic Lupus Erythematosus Activity Index-2 K.
*Data from Bergkamp, et al.1
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Key learnings |
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- Bergkamp SC, Kanagasabapathy T, Gruppen MP, et al. First validation of the childhood lupus low disease activity state (cLLDAS) definition in a real-life longitudinal cSLE cohort. Clin Immunol. 2024. Online ahead of print. DOI: 10.1016/j.clim.2024.110172
- Smith EMD, Aggarwal A, Ainsworth J, et al. PReS-endorsed international childhood lupus T2T task force definition of childhood lupus low disease activity state (cLLDAS). Clin Immunol. 2023;250:109296. DOI: 10.1016/j.clim.2023.109296
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