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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.
The new cLLDAS definition was retrospectively applied to prospectively collected data from the longitudinal, real-life cSLE cohort.
The objectives of the study were to:
compare the time to achieve first adult LLDAS (aLLDAS) vs cLLDAS;
Assess if patients with cSLE could maintain cLLDAS for ≥50% of follow-up time (cLLDAS-50); and
assess the predictors for cLLDAS-50 and occurrence of damage using univariate logistic regression analysis.
A total of 50 patients with cSLE were included; 86% were females and mean age at diagnosis was 14 years.
Each patient attained aLLDAS and cLLDAS at least once during their follow-up.
Mean time to attain first aLLDAS and cLLDAS was 8.2 and 9.0 months, respectively.
However, this difference was only due to one patient.
In 22 of the 42 patients, the mean delay to achieve first cLLDAS due to steroid dosage was 6.2 months.
Median time spent in first cLLDAS was 3 months, with 78% patients not remaining in first cLLDAS.
cLLDAS-50 was maintained in 58% of patients.
The predictors for attaining cLLDAS-50, as per univariate analysis, are depicted in Figure 1A.
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.
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.
The predictors of damage, as per univariate analysis, are depicted in Figure 1B.
The significance of these predictors for damage disappeared in the multivariate model.
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
Key learnings |
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References
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