title: Increased serum sFas, sTRAIL, and reduced sFasL in juvenile-onset systemic lupus erythematosus.
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.
authors.affiliation: Laboratory of Medical Investigation (LIM-36), Instituto da Criança, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar 647, 5° andar, LIM 36, Cerqueira Cesar, São Paulo, CEP 05403-900, Brazil. bernadete.liphaus@hc.fm.usp.br.
authors.forename: Bernadete L
authors.initials: BL
authors.lastname: Liphaus
authors.affiliation: Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Maria Helena B
authors.initials: MHB
authors.lastname: Kiss
authors.affiliation: Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Solange
authors.initials: S
authors.lastname: Carrasco
authors.affiliation: Laboratory of Medical Investigation (LIM-36), Instituto da Criança, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar 647, 5° andar, LIM 36, Cerqueira Cesar, São Paulo, CEP 05403-900, Brazil.
authors.forename: Patrícia
authors.initials: P
authors.lastname: Palmeira
authors.affiliation: Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Claudia
authors.initials: C
authors.lastname: Goldenstein-Schainberg
authors.affiliation: Laboratory of Medical Investigation (LIM-36), Instituto da Criança, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar 647, 5° andar, LIM 36, Cerqueira Cesar, São Paulo, CEP 05403-900, Brazil.
authors.forename: Magda
authors.initials: M
authors.lastname: Carneiro-Sampaio
mesh_terms: D000293:Adolescent; D002648:Child; D053222:Fas Ligand Protein / Q000097:blood*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood*; D008181:Lupus Nephritis / Q000097:blood; D008297:Male; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000097:blood; D053221:TNF-Related Apoptosis-Inducing Ligand / Q000097:blood*; D055815:Young Adult; D019014:fas Receptor / Q000097:blood*
chemical_list: C000595707:BCL2 protein, human; C491901:FAS protein, human; D053222:Fas Ligand Protein; D019253:Proto-Oncogene Proteins c-bcl-2; D053221:TNF-Related Apoptosis-Inducing Ligand; D019014:fas Receptor
keywords: Anti-dsDNA; Bcl-2; FasL; Juvenile-onset systemic lupus erythematosus; Nephritis; SLEDAI score; Soluble Fas; TRAIL