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Background Silent lupus nephritis (SLN) is systemic lupus erythematosus (SLE) without clinical and laboratory features of kidney involvement but with biopsy-proven nephritis. This study aims to describe and compare the baseline characteristics and outcomes of pediatric SLN with overt LN (OLN) and to identify associated risk factors and biochemical markers. Methods In this retrospective, observational study, multivariate logistic regression and receiver operating characteristic (ROC) analyses studied age, sex, race, serum complements, anti-double-stranded-DNA antibody, anti-Smith antibody, eGFR, and proliferative nephritis. Results In our cohort of 69 patients, 47 were OLN, and 22 were SLN. OLN (OR = 4.9, p = 0.03) and non-African Americans (AA) (OR = 13.0, p OR 0.95 and 0.65 per one unit increase in C3 and C4, respectively, p Conclusions Lower complement levels are associated with proliferative lesions in pediatric LN—both SLN and OLN. The non-AA population had higher odds of having proliferative nephritis than the AA. Prospective, randomized, long-term follow-up of proliferative SLN patients is needed to ascertain the beneficial effect of early diagnosis and treatment. Graphical abstract

Systemic lupus erythematosus (SLE) is an autoimmune disorder with significant health disparities, as it disproportionately and more severely affects vulnerable and disadvantaged population groups in the United States and around the world, that is, women, ethnic minorities, individuals living in poverty, less educated, and lacking medical insurance. Both, genetic and non-genetic factors, contribute to these disparities. To overcome these health disparities and reduce poor outcomes among disadvantaged SLE populations, interventions on non-genetic amendable factors, especially on social health determinants, are necessary., Fil: González, Luis A. Universidad de Antioquia. School of Medicine. Department of Internal Medicine. Division of Rheumatology; Colombia, Fil: Ugarte-Gil, Manuel F. Hospital Nacional Guillermo Almenara Irigoyen; Perú, Fil: Ugarte-Gil, Manuel F. Universidad Científica Del Sur. Grupo Peruano de Estudio de Enfermedades Autoimmunes Sistémicas. Perú, Fil: Pons-Estel, Guillermo J. Grupo Oroño - Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR); Argentina, Fil: Durán-Barragán, Sergio. Universidad de Guadalajara. Departamento de Clínicas Médicas. Instituto de Investigación en Reumatología y Del Sistema Musculoesquelético; México, Fil: Durán-Barragán, Sergio. Clínica de Investigación en Reumatología y Obesidad S.C; México, Fil: Toloza, Sergio. Hospital San Juan Bautista. Rheumatology Unit. Department of Medicine; Argentina, Fil: Burgos, Paula I. Pontificia Universidad Católica de Chile. School of Medicine. Department of Clinical Immunology and Rheumatology; Chile, Fil: Bertoli, Ana. Universidad Católica de Córdoba. Clínica Universitaria Reina Fabiola. Sevicio de Reumatología; Argentina, Fil: Borgia, R Ezequiel. University of Florida. College of Medicine. Immunology and Rheumatology. Division of Allergy. Department of Pediatrics; Estados Unidos, Fil: Borgia, R Ezequiel. University of Florida. College of Medicine. Department of Health Outcomes and Biomedical Informatics; Estados Unidos, Fil: Alarcón, Graciela S. Heersink School of Medicine. The University of Alabama at Birmingham. Department of Medicine. Division of Clinical Immunology and Rheumatology; Estados Unidos, Fil: Alarcón, Graciela S. Universidad Peruana Cayetano. School of Medicine. Department of Medicine; Perú

The long-term impact of childhood-onset systemic lupus erythematosus on Health-related Quality of Life (HRQoL) in adult SLE patients in comparison to those with adult-onset SLE is unknown. We aim to examine and compare HRQoL trajectories in adults with adolescent- and adult-onset SLE.Patients enrolled in the LUMINA cohort were included. Adolescent-onset SLE were those diagnosed24 years of age, and adult-onset SLE otherwise. Sociodemographic, clinical, medications, behavioral/psychological and functioning data were obtained. Longitudinal trajectories of the physical component (PCS) and the mental component (MCS) SF-36 summary scores were compared between the groups using a linear mixed model accounting for time-dependent and independent covariates.470 SLE patients were included (95 adolescent-onset SLE and 375 adult-onset SLE). The mean age at diagnosis was 19.7 years (SD: 2.8) in the adolescent group and 39.3 years (SD: 11.0) in the adult group. Baseline PCS was higher (better physical functioning) in adolescent- when compared to adult-onset SLE (38.9 vs 34.3 respectively, p0.001); however, the baseline MCS scores was comparable between the groups (41.4 vs 40.5 respectively, p=0.53). The HRQoL improved equally in both groups with no statistically significant difference within and between the groups (last PCS and MCS mean scores: 43.9 and 45.3 in adolescent-onset SLE; 38.1 and 43 in adult-onset SLE respectively).Adults with adolescent-onset SLE exhibited better physical functioning when compared with the adult SLE group despite more severe disease; noteworthy, HRQoL was below the general US population despite clinically meaningful improvement in HRQoL over time in both groups. This article is protected by copyright. All rights reserved.

This study assesses the association of race/ethnicity with the Patient-Reported Outcomes Measurement Information System (PROMIS®) in childhood-onset systemic lupus erythematosus (cSLE) patients from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.cSLE patients enrolled in the CARRA Registry within two years of cSLE diagnosis who met ACR and/or SLICC classification criteria for lupus were included. Baseline demographics, laboratory, and disease features as well as patient-reported outcomes were obtained. Multivariable linear regression analysis was used to examine the association of race and ethnicity with PROMIS scores at registry enrolment.425 cSLE patients met inclusion criteria: 83.8% were female, 30.6% non-Hispanic White, 29.7% Black, 22.1% Hispanic. The mean age at diagnosis was 13.9 years (SD 2.5). Household income and highest parental education varied by race/ethnic group, as did frequency of rash, leukopenia, and anti-Smith antibodies. The cohort had low-moderate baseline disease activity (SLEDAI mean: 6.0 [SD 6.7]). The overall PROMIS Global Health mean T-score was 38.6 (SD 6.5), more than one standard deviation below the general population mean of 50. There was no association between race/ethnicity and PROMIS scores in multivariable linear regression analysis.In this multiethnic paediatric lupus cohort, PROMIS global health was lower when compared with the general paediatric US population. Moreover, PROMIS global health, pain interference, and physical function mobility did not vary across races/ethnicities.

Background The association of race/ethnicity with childhood-onset systemic lupus erythematosus (cSLE) outcomes has been well described, with non-White individuals experiencing a more severe disease phenotype including increased damage accrual and higher rates of renal involvement. However, there are limited data regarding patient-reported outcomes (PROs) across race and ethnic groups in cSLE. This study assesses the association of race/ethnicity with Patient-Reported Outcomes Measurement Information System (PROMIS®) in cSLE patients at enrollment in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Methods The CARRA Registry is a convenience cohort of pediatric patients with rheumatic disease including cSLE primarily from North America. This study included cSLE patients enrolled in the CARRA registry within two years from cSLE diagnosis who met ≥ 4/11 ACR classification criteria for lupus. Baseline demographics, laboratory, and disease features as well as PROs were obtained. Analysis of variance (ANOVA) was used to assess the difference in the PROMIS summary T-score means across races/ethnicities. Relationships between SLEDAI and PRO scores were analyzed using Pearson’s correlation coefficients. Multivariable linear regression analysis was used to examine the association of race and ethnicity with PROMIS scores at baseline. P-values Results 588 cSLE patients met inclusion criteria: 84% were female, 24.2% non-Hispanic White, 23% African American, and 19% Hispanic. The mean age at diagnosis was 13.2 years (SD 3.1). Household income and highest parental education varied by race/ethnic group, as did frequency of rash, leukopenia, and positive anti-Sm antibody (table 1). The cohort had low-moderate baseline disease activity, with a mean SLEDAI of 6.14 (SD 6.8). There was no difference in disease activity across races/ethnicities in one-way ANOVA analysis. The overall PROMIS PHG-7 mean T-score was 38.6 (SD 6.4), more than 1 SD below the general population mean of 50. The mean Pain Interference T-score of 54 (SD 10) was slightly above, and the Physical Function Mobility mean T-score of 47.5 (SD 10.13) was slightly below the population mean of 50. There was a negative moderate correlation between PROMIS Pain Interference and Physical Function Mobility (Pearson correlation coefficient: −0.52). There was no association between race/ethnicity and PROMIS scores in multivariable linear regression analysis. Conclusions In this large multiethnic CARRA pediatric lupus cohort, global health (PGH-7), pain interference, and physical function mobility as measured by PROMIS did not vary across races/ethnicities. The relatively low baseline disease activity may explain this lack of association. Long-term follow-up data is needed to assess for associations between PROs and race/ethnicity over time.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by evidence of autoantibodies and multiorgan system involvement leading to significant physical and functional disability. The reported incidence and prevalence of SLE in the US vary by region but are estimated to be approximately 5.5 per 100,000 persons per year and 72.8 per 100,000 persons, respectively. Several social determinants of health, including educational level, health insurance, household income, and social support, as well as environmental and occupational exposures may impact lupus outcomes. The association of race/ethnicity with SLE outcomes has also been reported, with non White minorities experiencing a more severe disease phenotype with increased damage accrual and higher rates of renal involvement when compared to White individuals. The goals of Healthy People 2020, a set of national public health objectives released by the US Department of Health and Human Services, include achieving health equity, eliminating health disparities, and overall improving the health of the nation’s population through collaboration among diverse groups. Socioeconomic inequalities are a common cause of health disparities; therefore, addressing social determinants of health would enable health professionals to enhance prevention approaches and health promotions that reduce health inequities. In this editorial, we highlight several Community-Based Participatory Research (CBPR) principles and potential applications to address health disparities in SLE...

ObjectiveMacrophage activation syndrome (MAS), a life-threatening inflammatory complication, is increasingly recognized in childhood-onset systemic lupus erythematosus (cSLE). It can be a challenge to differentiate active cSLE from MAS. We generated decision rules for discriminating MAS from active cSLE in newly diagnosed patients.MethodsWe conducted a retrospective cohort study of consecutive, newly diagnosed, active cSLE patients with fever, requiring hospital admission to The Hospital for Sick Children from January 2003 to December 2007 (cohort 1) and January 2008 to December 2013 (cohort 2). All patients met ≥ 4 American College of Rheumatology or Systemic Lupus International Collaborating Clinics criteria, and were steroid-naïve and infection-free. MAS was diagnosed based on expert opinion. Recursive partitioning was applied to each cohort to derive a decision rule based on clinical and laboratory features, distinguishing MAS from non-MAS cSLE. Each decision rule was applied to the alternate, independent cohort. Sensitivity and specificity of these decision rules were compared to existing criteria.ResultsCohort 1 (n = 34) and cohort 2 (n = 41) each had 10 patients with MAS. Recursive partitioning in cohort 1 identified ferritin ≥ 699 μg/L as the sole best discriminator between MAS and non-MAS patients (R2 = 0.48), and in cohort 2, ferritin ≥ 1107 μg/L was the best discriminator for MAS, followed by lymphocytes < 0.72 × 103/mm3 (R2 = 0.52). Cross-validation of our decision rules maintained 90–100% sensitivity and 65–85% specificity.ConclusionOur decision rule demonstrated improved performance compared to preliminary guidelines for MAS in cSLE from the Lupus Working Group of the Paediatric Rheumatology European Society and familial hemophagocytic lymphohistiocytosis diagnostic criteria. Validation in independent cohorts is required.