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Validation of childhood lupus low disease activity state definition

By Haimanti Mandal

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Apr 9, 2024

Learning objective: After reading this article, learners will be able to cite a new clinical development in systemic lupus erythematosus.

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 

  • 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. 

Key findings1 

  • A total of 50 patients with cSLE were included; 86% were females and mean age at diagnosis was 14 years. 

cLLDAS 

  • 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 

  • 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 

  • 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
  • Time to reach cLLDAS differs from time to reach aLLDAS in cSLE, which validates the new cLLDAS definition.
  • Achieving cLLDAS is a feasible treatment goal in cSLE; however, attaining cLLDAS-50 appears challenging.
  • Biologics (if available) should be initiated earlier, especially in patients with cSLE with an African/Afro-Caribbean ethnic background, higher SLEDAI at diagnosis, and those who exhibit damage within first year after diagnosis.
  • Further treat-to-target trials are needed in patients with cSLE to gather more evidence on early steroid-tapering and initiating steroid-sparing drugs for preventing damage in cSLE.

References

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