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Increased serum sFas, sTRAIL, and reduced sFasL in juvenile-onset systemic lupus erythematosus

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Abstract

The aims of this study were to assess serum Fas, FasL, TRAIL, and Bcl-2 levels in patients with juvenile-onset systemic lupus erythematosus (JSLE) and to evaluate their relations with disease activity parameters and nephritis. Forty-eight JSLE patients, 33 juvenile idiopathic arthritis (JIA, inflammatory controls) patients and 40 healthy controls were enrolled. sFas, sFasL, sTRAIL, and sBcl-2 serum levels were measured by ELISA. Disease activity parameters included SLEDAI score, ESR, anti-dsDNA antibodies, C3, and C4 levels. Thirty-five JSLE patients had nephritis and 32 patients were classified as having active disease (SLEDAI ≥4). Statistical analysis methods included Mann-Whitney test and Spearman’s rank test. JSLE patients had significantly increased sFas serum levels compared with healthy controls (median 177.6 vs. 117.5 pg/mL; p = 0.0001), higher sTRAIL (median 484.6 vs 270.8 pg/mL; p = 0.02), and reduced sFasL (median 0.05 vs 0.3 ng/mL; p = 0.0002). The same results were observed for JSLE patients with active disease and for patients with nephritis. Additionally, sFas levels in JSLE patients directly correlated with SLEDAI score (r = 0.40; p = 0.009), and sTRAIL levels were increased in JSLE patients with neuropsychiatric disease compared with those without this involvement (median 667.9 vs. 216.2 pg/mL; p = 0.03). Otherwise, sBcl-2 levels of JSLE patients were similar to healthy controls. JIA patients had sFas, sFasL, sTRAIL, and sBcl-2 serum levels similar to JSLE patients and to healthy controls. In summary, this study characterized in JSLE a distinct profile from adult SLE that comprises increased sFas, sTRAIL, and reduced sFasL, notably in patients with active disease and with nephritis.

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Acknowledgments

This study was partially supported by FAPESP (Fundação de Amparo à Pesquisa do Estado de São Paulo) grant no. 2008/58238-4. Authors thank all patients for their kind participation and Dr. Clovis A. Silva for enabling patients’ enrollment. All authors were involved in drafting or revising the manuscript, and approved final version.

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Correspondence to Bernadete L. Liphaus.

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Liphaus, B.L., Kiss, M.H.B., Carrasco, S. et al. Increased serum sFas, sTRAIL, and reduced sFasL in juvenile-onset systemic lupus erythematosus. Clin Rheumatol 36, 2847–2852 (2017). https://doi.org/10.1007/s10067-017-3615-8

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