Skip to main content
  • Poster presentation
  • Open access
  • Published:

B lymphocyte stimulator, interferon-α and HMGB 1 interrelation in childhood onset systemic lupus erythematosus: associations with disease activity and severity


The role of B lymphocyte stimulator (Blys) in Childhood onset Systemic Lupus Erythematosus (cSLE) has not been elucidated.


To investigate the role of Blys and its association with biomarkers related with the cSLE disease activity and severity.


34 cSLE patients (study group), 51 with other rheumatic or autoimmune diseases and 26 healthy children (control groups) were studied. For evaluating the disease activity, SLEDAI and ECLAM scores were used. For estimating the disease severity the criteria were: involvement of 3 vital organs (kidneys or CNS or blood or combination of ≥2 organs), increased anti-dsDNA titres, low C3 /C4 concentrations and high concentrations of serum IFN-α and HMGB1 (High mobility group box 1) protein.


Mean Blys levels were significantly higher in cSLE patients in respect to patients of all control groups, in patients with active as compared with patients with inactive disease and in patients with vital organ involvement as compared with patients without such involvement. Moreover, mean Blys levels were positively correlated with anti-DNA titer, IFN-α and HMGB1 levels but negatively correlated with C3 /C4 concentrations.


Taking together, results of our study indicate that: high levels of Blys are mainly found in serum of jSLE patients and they may be involved in the increased anti-DNA plus other autoantibody production. Moreover, they are associated with the disease activity and severity, meaning that Blys is a specific biomarker for the selection of patients who will need more aggressive and possibly more targeted than conventional therapy.

Disclosure of interest

None declared

Author information

Authors and Affiliations


Rights and permissions

Open Access  This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made.

The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder.

To view a copy of this licence, visit

The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kanakoudi-Tsakalidou, F., Tzimouli, V., Farmaki, E. et al. B lymphocyte stimulator, interferon-α and HMGB 1 interrelation in childhood onset systemic lupus erythematosus: associations with disease activity and severity. Pediatr Rheumatol 12 (Suppl 1), P321 (2014).

Download citation

  • Published:

  • DOI: