Search

Objective Pediatric systemic lupus erythematosus (SLE) is a chronic fluctuating disease that significantly impacts quality of life (QOL). There is no pediatric SLE-specific health-related QOL (HRQOL) scale. Our objective was to develop and validate a new pediatric SLE-specific HRQOL scale. Methods We developed the Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY) based on results of qualitative research of children with SLE and their parents. SMILEY has parallel child and parent reports with a 5-faces scale for responses. SMILEY comprises 4 domains: effect on self, limitations, social, and burden of SLE. In this cross-sectional study, we examined face, content, construct, and concurrent validity; internal consistency; test–retest reliability; and child-parent agreement for SMILEY. Children ≤18 years of age with SLE and their parents completed corresponding child and parent SMILEY reports, as well as other QOL and physical function scales. Qualified physicians assessed SLE activity, damage, and severity. Results Eighty-six children with SLE and 80 parents participated. SMILEY was found to have face, content, construct, and concurrent validity (Spearman's rank correlation [rs] ≥0.4); test–retest reliability (intraclass correlation coefficient [ICC] 0.9); and internal consistency (α = 0.9). Moderate agreement was found between child and parent SMILEY reports (ICC 0.7, rs = 0.5, P < 0.001). Conclusion SMILEY is a brief, easily understood, valid, and reliable pediatric SLE-specific QOL scale. Because SMILEY assesses children's self-perception of QOL as impacted by SLE, we predict that it will have great utility in clinical practice, clinical trials, and outcomes research.

Objective Gene-expression studies have demonstrated increased expression of interferon (IFN)–inducible genes (IFIGs) in peripheral blood mononuclear cells (PBMCs) of many patients with systemic lupus erythematosus (SLE), with a predominant effect of type I IFN. This study examined the hypothesis that increased disease severity and activity, as well as distinct autoantibody specificities, characterize SLE patients with activation of the type I IFN pathway. Methods Freshly isolated PBMCs from 77 SLE patients, 22 disease controls, and 28 healthy donors were subjected to real-time polymerase chain reaction for 3 IFIGs that are preferentially induced by IFNα, and the data were used to derive IFNα scores for all individuals. Expression of IFIGs was significantly higher in SLE patients compared with disease controls or healthy donors. SLE patients with high and low IFNα scores were compared for clinical manifestations of disease, disease severity, disease activity, serologic features, and potential confounders, by bivariate and multivariate analyses. Results SLE patients with a high IFNα score had a significantly higher prevalence of renal disease, a greater number of American College of Rheumatology criteria for SLE, and a higher Systemic Lupus International Collaborating Clinics damage index (SDI) score than did SLE patients with low IFNα scores. Patients with high scores showed increased disease activity, as measured by lower C3 levels, hemoglobin levels, absolute lymphocyte counts, and albumin levels, and a higher anti–double-stranded DNA (dsDNA) titer, erythrocyte sedimentation rate, and SLE Disease Activity Index 2000 score. The presence of antibodies specific for Ro, U1 RNP, Sm, and dsDNA, but not phospholipids, was significantly associated with a high IFNα score. Logistic regression analysis confirmed that renal disease, higher SDI scores, low complement levels, and presence of anti–RNA binding protein (RBP) autoantibodies were associated with a high IFNα score. Conclusion Activation of the IFNα pathway defines a subgroup of SLE patients whose condition is characterized by increased disease severity, including renal disease, increased disease activity, reflected in complement activation, and autoreactivity to RBP.

Objective To study the contribution of interferon-α (IFNα) and IFNγ to the IFN gene expression signature that has been observed in microarray screens of peripheral blood mononuclear cells (PBMCs) from patients with systemic lupus erythematosus (SLE). Methods Quantitative real-time polymerase chain reaction analysis of healthy control PBMCs was used to determine the relative induction of a panel of IFN-inducible genes (IFIGs) by IFNα and IFNγ. PBMCs from 77 SLE patients were compared with those from 22 disease controls and 28 healthy donors for expression of IFIGs. Results Expression of IFNα-inducible genes was significantly higher in SLE PBMCs than in those from disease controls or healthy donors. The level of expression of all IFIGs in PBMCs from SLE patients with IFNα pathway activation correlated highly with the inherent responsiveness of those genes to IFNα, suggesting coordinate activation of that cytokine pathway. Expression of genes preferentially induced by IFNγ was not significantly increased in SLE PBMCs compared with control PBMCs. IFNα-regulated gene-inducing activity was detected in some SLE plasma samples. Conclusion The coordinate activation of IFNα-induced genes is a characteristic of PBMCs from many SLE patients, supporting the hypothesis that IFNα is the predominant stimulus for IFIG expression in lupus.

Objective To determine whether social network experience and perceptions of benefit of arthritis treatments influence the decision to seek diagnosis and treatment. Methods A population-based telephone survey of 515 black and 455 white Medicare beneficiaries was conducted. Validated questionnaires adapted for use in a telephone interview were used to identify people with self-reported symptoms of hip or knee pain. Treatment history for arthritis-related pain and perceptions of benefit of treatment were also assessed. Results Forty-two percent of blacks and 31% of whites reported hip or knee pain. Forty-two percent of blacks and 65% of whites reported knowing someone who had surgery for hip or knee pain (P < 0.0001). Blacks were less likely than whites to report that surgery had helped someone they knew with hip or knee pain (not significant). Conclusion Blacks know fewer people who have had surgical treatment of hip and knee pain than whites and appear to be less likely to perceive that such treatment is beneficial.