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  • Open Access

PReS-FINAL-2352: Apoptosis profile in patients with juvenile-onset systemic lupus erythematosus

  • B Liphaus1,
  • MH Kiss1,
  • S Carrasco1,
  • C Goldenstein-Schainberg1 and
  • Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil
Pediatric Rheumatology201311(Suppl 2):P342

https://doi.org/10.1186/1546-0096-11-S2-P342

Published: 5 December 2013

Keywords

Systemic Lupus ErythematosusMean Fluorescence IntensityImmune DysregulationApoptosis Related ProteinSoluble Molecule

Introduction

Apoptosis related proteins have been involved in immune dysregulation and development of systemic lupus erythematosus (SLE).

Objectives

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.

Methods

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.

Results

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.

Conclusion

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

None declared.

Authors’ Affiliations

(1)
Reumatologia, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil

Copyright

© Liphaus et al.; licensee BioMed Central Ltd. 2013

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.

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