- Poster presentation
- Open Access
PReS-FINAL-2352: Apoptosis profile in patients with juvenile-onset systemic lupus erythematosus
Pediatric Rheumatology volume 11, Article number: P342 (2013)
Apoptosis related proteins have been involved in immune dysregulation and development of systemic lupus erythematosus (SLE).
To assess sFas, sFasL, sTRAIL and sBcl-2 in sera and to evaluate Fas and Bcl-2 expressions in peripheral monocytes, T and B lymphocytes from juvenile-onset SLE (JSLE) and to determine relationships with disease activity.
Forty-three JSLE patients (revised ACR criteria, mean age = 14.3 yrs, 36F:7M), and 35 age and gender matched healthy controls were studied; 30 JSLE had SLEDAI score3 4, reflected active disease. Soluble molecules were measured by commercial ELISA kits. Lymphocytes and monocytes were stained with specific moAbs and analyzed by flow cytometry. Kruskal-Wallis test and Spearman's rank were employed and statistical significance considered p value < 0.05.
JSLE sera had significantly increased sFas (188.1 ± 69.2 vs 133.2 ± 80.6, pg/ml) and sTRAIL (691.3 ± 631.8 vs 346.6 ± 251.1, pg/ml), decreased sFasL (0.08 ± 0.1 vs 0.36 ± 0.4, ng/ml), and similar sBcl-2 (7.4 ± 8.6 vs 9.3 ± 9.6, mg/ml) levels compared to healthy controls. SLEDAI score directly correlated with sFas (r = 0.52; p = 0.001). JSLE patients compared to controls had significantly increased Fas expression on CD3+ (43.7 ± 10.3% vs 28.9 ± 9.4%), CD4+ (20.3 ± 6.7% vs 16.2 ± 6.2%) and CD8+ (21.5 ± 9.6% vs 12.3 ± 5.8%) T cells, and also on CD19+ B cells (2.1 ± 1.4% vs 1.4 ± 0.7%), whereas, it was decreased on CD14+ monocytes (93.6 ± 6.9% vs 96.7 ± 2.5%, p = 0.01). There was direct correlation between percentages of CD19+Fas+ cells and SLEDAI (r = 0.38, p = 0.02) and inverse correlation between percentages of CD14+Fas+ cells and SLEDAI (r= -0.55, p = 0.01). Mean fluorescence intensity (MFI) of Bcl-2-positive cells from JSLE patients was significantly increased in CD3+ (28.8 ± 8.4 vs 22.9 ± 4.2), CD4+ (28.6 ± 8.2 vs 22.9 ± 4.4) and CD8+ (29.4 ± 9.4 vs 22.8 ± 3.6) T cells, and also in CD19+ B cells (25.5 ± 9.6 vs 21.5 ± 3.6). Bcl-2 expression in CD14+ monocytes was lower in JSLE compared to controls (25.2 ± 18.2% vs 34.5 ± 16.6%, p = 0.006). Direct correlation between percentages of CD19+Bcl-2+ cells and SLEDAI (r = 0.47, p = 0.04) was shown.
JSLE patients showing high sFas and sTRAIL and low sFasL levels with Fas and Bcl-2 expressions increased on circulating T and B lymphocytes though decreased on monocytes are remarkable evidences of apoptosis role in the immune dysregulation observed. A possible role as a marker for lupus disease activity needs to be defined.
Disclosure of interest
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
About this article
Cite this article
Liphaus, B., Kiss, M., Carrasco, S. et al. PReS-FINAL-2352: Apoptosis profile in patients with juvenile-onset systemic lupus erythematosus. Pediatr Rheumatol 11 (Suppl 2), P342 (2013). https://doi.org/10.1186/1546-0096-11-S2-P342
- Systemic Lupus Erythematosus
- Mean Fluorescence Intensity
- Immune Dysregulation
- Apoptosis Related Protein
- Soluble Molecule